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Amraie M, Khorraminejad-Shirazi M, Nabavizadeh A, Mokhtari M. Evaluation of Diagnostic Accuracy of Immunohistochemical Markers of SATB2 and Villin in Differential Diagnosis of Ovarian and Gastrointestinal Mucinous Carcinoma. Appl Immunohistochem Mol Morphol 2025:00129039-990000000-00227. [PMID: 40400355 DOI: 10.1097/pai.0000000000001265] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2024] [Accepted: 04/17/2025] [Indexed: 05/23/2025]
Abstract
Determining the origin of mucinous adenocarcinomas can be challenging due to overlapping histologic and immunohistochemical features. This study evaluates the utility of SATB2 and villin immunohistochemical markers for defining mucinous adenocarcinomas' origin. We retrospectively analyzed 71 patients with mucinous adenocarcinomas-31 lower gastrointestinal (GI), 28 ovarian, and 12 cases of pseudomyxoma peritonei. Immunohistochemistry for SATB2 and villin was performed on ovarian and GI tumor samples. Sensitivity, specificity, and positive and negative predictive values were calculated to assess diagnostic performance.For identifying GI origin, SATB2 showed 87.1% sensitivity and 100% specificity, while villin exhibited 93.5% sensitivity but only 21.4% specificity. Dual SATB2/villin positivity demonstrated 80.6% sensitivity and 100% specificity for GI origin. For ovarian origin, dual SATB2/villin negativity provided 100% specificity. Logistic regression analysis on the adenocarcinoma cases showed 93.2% accuracy rate of dual staining with SATB2 and villin for predicting GI versus ovarian origin. SATB2 exhibits high specificity for GI mucinous adenocarcinomas, and villin is highly sensitive. Combining SATB2 and villin optimizes diagnostic performance for differentiating the primary origin site of mucinous adenocarcinomas and determining pseudomyxoma peritonei origin. Moreover, the presence of signet ring morphology prompted reconsideration of possible GI origin among our cases. A multimarker immunohistochemical panel, including SATB2 and villin can enhance the diagnostic accuracy of challenging mucinous adenocarcinoma cases.
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Affiliation(s)
- Mehrjoo Amraie
- Department of Pathology, School of Medicine, Shiraz University of Medical Sciences
| | - Mohammadhossein Khorraminejad-Shirazi
- Department of Pathology, School of Medicine, Shiraz University of Medical Sciences
- Student research committee, Shiraz University of Medical Sciences
- Department of Pathology, School of Medicine, Jahrom University of Medical Sciences, Jahrom, Iran
| | - Ali Nabavizadeh
- Otolaryngology Research Center, Department of Otolaryngology, Shiraz University of Medical Sciences
| | - Maral Mokhtari
- Department of Pathology, Shahid Faghihi hospital, School of Medicine, Shiraz University of Medical Sciences
- Colorectal Research center, Shiraz University of Medical Sciences
- Breast Research center, Shiraz University of Medical Sciences, Shiraz, Iran
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Ha YJ, Park SH, Kim SK, Tak KH, Kim JH, Kim CW, Yoon YS, Kim SY, Lee JL. Molecular characterization of Pseudomyxoma peritonei with single-cell and bulk RNA sequencing. Sci Data 2025; 12:213. [PMID: 39910051 PMCID: PMC11799422 DOI: 10.1038/s41597-025-04561-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2024] [Accepted: 01/30/2025] [Indexed: 02/07/2025] Open
Abstract
Pseudomyxoma peritonei (PMP), a rare condition characterized by mucinous ascites in the peritoneal cavity, often leads to a poor prognosis. However, omics profiling of this disease remains significantly underexplored. Here, we present single-cell transcriptomic profiling of five PMP cases to identify cell type-specific gene features associated with PMP pathogenesis. Additionally, we provide bulk RNA-seq datasets from two independent cohorts: 19 fresh frozen tissue samples (12 PMPs) and 34 formalin-fixed paraffin-embedded (FFPE) samples (25 PMPs). We also offer protein expression data from a tissue microarray (TMA) analysis of 90 samples (45 PMPs). Our single-cell and bulk transcriptomic profiles, along with TMA verifications, reveal the cellular diversity of PMP, highlighting the coexistence of epithelial and mesenchymal characteristics within PMP cells. These datasets enhance our understanding of PMP pathogenesis and provide a valuable resource for uncovering the intricate molecular landscape of PMP, with the potential to improve clinical utility through further research.
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Affiliation(s)
- Ye Jin Ha
- Asan Institute for Life Sciences, Asan Medical Center, Seoul, 05505, Korea
| | - Seong-Hwan Park
- Aging Convergence Research Center, Korea Research Institute of Bioscience and Biotechnology (KRIBB), Daejeon, 34141, Korea
- Department of Bioscience, University of Science and Technology, Daejeon, 34113, Korea
| | - Seon-Kyu Kim
- Aging Convergence Research Center, Korea Research Institute of Bioscience and Biotechnology (KRIBB), Daejeon, 34141, Korea
- Department of Bioscience, University of Science and Technology, Daejeon, 34113, Korea
- Personalized Genomic Medicine Research Center, KRIBB, Daejeon, 34141, Korea
| | - Ka Hee Tak
- Asan Institute for Life Sciences, Asan Medical Center, Seoul, 05505, Korea
| | - Jeong-Hwan Kim
- Aging Convergence Research Center, Korea Research Institute of Bioscience and Biotechnology (KRIBB), Daejeon, 34141, Korea
- Personalized Genomic Medicine Research Center, KRIBB, Daejeon, 34141, Korea
| | - Chan Wook Kim
- Asan Institute for Life Sciences, Asan Medical Center, Seoul, 05505, Korea
- Division of Colon and Rectal Surgery, Department of surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul, 05505, Korea
| | - Yong Sik Yoon
- Asan Institute for Life Sciences, Asan Medical Center, Seoul, 05505, Korea
- Division of Colon and Rectal Surgery, Department of surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul, 05505, Korea
| | - Seon-Young Kim
- Department of Bioscience, University of Science and Technology, Daejeon, 34113, Korea.
- Personalized Genomic Medicine Research Center, KRIBB, Daejeon, 34141, Korea.
- Korea Bioinformation Center, KRIBB, Daejeon, 34141, Korea.
| | - Jong Lyul Lee
- Asan Institute for Life Sciences, Asan Medical Center, Seoul, 05505, Korea.
- Division of Colon and Rectal Surgery, Department of surgery, University of Ulsan College of Medicine, Asan Medical Center, Seoul, 05505, Korea.
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Mousa AH, Nukaly HY, Samman RR, Fuadah S, Saddiq BWB, Alshowaikhat SJ, Khalid I. Pseudomyxoma peritonei of appendiceal mucinous neoplasm origin: A case report and review of literature. Radiol Case Rep 2024; 19:6565-6573. [PMID: 39391033 PMCID: PMC11465061 DOI: 10.1016/j.radcr.2024.08.158] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2024] [Revised: 08/28/2024] [Accepted: 08/29/2024] [Indexed: 10/12/2024] Open
Abstract
Appendiceal mucinous neoplasms, a rarity comprising less than 1% of all cancers, present intricate challenges in clinical management, and their incidence is on the rise. Notably, these neoplasms tend to metastasize intraperitoneally, leading to peritoneal carcinomatosis and concurrent accumulation of mucinous material, resulting in pseudomyxoma peritonei. Due to its spectrum of presentation, the classification of the appendiceal mucinous neoplasms remains a controversial subject with a range of management from a simple appendicectomy to a complex hyperthermic intraperitoneal chemotherapy (HIPEC). A 42-year-old Chadian male presented to the hospital with a sudden onset of right lower abdominal pain radiating to the inguinal region for 24 hours, associated with nausea and vomiting. The abdomen was distended and ascitic. Laboratory investigations revealed anemia, leukocytosis, hypernatremia, hypokalemia, elevated ESR, high CEA marker, and normal CA19-9. An abdominopelvic CT with contrast demonstrated extensive ascites and cystic masses in the liver, and pancreas with soft tissue thickening of the cecum; however, the appendix is not well-delineated. Patient was managed with chemotherapy and HIPEC followed by removal of all the affected parts. Nodules of the peritoneum and liver were submitted for histopathological analysis and a final diagnosis of pseudomyxoma peritonei of primary appendicular origin was established. This case highlights a case of extensive pseudomyxoma peritonei of appendicular origin managed aggressively by HIPEC and multiple resections of the involved organs. Prognosis of such a case is determined by the grade of the appendiceal tumor and the extent of invasion.
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Affiliation(s)
- Ahmed Hafez Mousa
- Department of Neurosurgery, Graduate Medical Education Department (GME), Mohammed Bin Rashid University of Medicine and Health Sciences (MBRU), Dubai Health, Dubai, United Arab Emirates
| | - Houriah Yasir Nukaly
- College of Medicine and Surgery, Batterjee Medical College, Jeddah, Saudi Arabia
| | - Rayyan Rafat Samman
- College of Medicine and Surgery, Batterjee Medical College, Jeddah, Saudi Arabia
| | - Samratul Fuadah
- College of Medicine and Surgery, Batterjee Medical College, Jeddah, Saudi Arabia
| | | | | | - Islam Khalid
- Department of Surgery, Faculty of Medicine, Suez Canal University Hospitals, Ismailia, Egypt
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Berton GG, Volino GC, de Britto GD, Guerra GC, Júnior JP, Corrêa NB, Taniguchi JB, Comarú LM, Biesek L, Bueno JPC, Lüdtke IN, Marcolan AM, Morassutti AL. Hydatid cysts in the mesocolon mimicking peritoneal pseudomyxoma in a post-appendectomy patient: A case report. Diagn Microbiol Infect Dis 2024; 110:116496. [PMID: 39244842 DOI: 10.1016/j.diagmicrobio.2024.116496] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2024] [Revised: 08/13/2024] [Accepted: 08/13/2024] [Indexed: 09/10/2024]
Abstract
We aimed to present a case of two mesocolonic hydatid cysts that mimicked the presentation of peritoneal pseudomyxoma. Hydatidosis is a zoonotic parasitic infection caused by the cestode Echinococcus spp., whose larval stage affects various organs. The present case describes a 40-year-old male patient who presented with severe lower abdominal pain and was diagnosed with acute appendicitis. The patient underwent an appendectomy and was later referred to an oncology surgery clinic because of imaging findings suggestive of peritoneal pseudomyxoma or carcinomatosis. A video-assisted laparoscopic procedure revealed two cysts and microscopic findings confirmed hydatid cysts. The patient was from a hydatidosis-endemic region of southern Brazil. This case highlights the diagnostic challenges and the need for a multidisciplinary approach and careful histopathological analysis in patients with complex abdominal conditions. This also demonstrates the importance of disseminating knowledge about this condition and its management.
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Affiliation(s)
- Giovanni Gosch Berton
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil; School of Medicine, Università degli Studi di Padova, Ospedale Civile St., 77, Padua, Veneto, Italy
| | - Giovanni Cândido Volino
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Gabriel Diehl de Britto
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Giancarlo Canello Guerra
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Juliano Poleze Júnior
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Nathalia Beck Corrêa
- General Surgery, Santa Casa de Misericórdia de Porto Alegre, Professor Annes Dias St., 295, Porto Alegre, Rio Grande do Sul, Brazil
| | - Juliana Bosso Taniguchi
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Luísa Motter Comarú
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Laura Biesek
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Júlia Papaleo Costa Bueno
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil
| | - Ivan Neutzling Lüdtke
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil; Oncological Surgery, Hospital de Clínicas de Carazinho, General Câmara St., 70, Carazinho, Rio Grande do Sul, Brazil
| | - Ana Maria Marcolan
- Pathology Institute of Passo Fundo, XV de Novembro St., 328, Passo Fundo, Rio Grande do Sul, Brazil
| | - Alessandra Loureiro Morassutti
- School of Medicine, Universidade de Passo Fundo, Teixeira Soares St., 817, Passo Fundo, Rio Grande do Sul, Brazil; Pathology Institute of Passo Fundo, XV de Novembro St., 328, Passo Fundo, Rio Grande do Sul, Brazil; School of Dentistry, University of Passo Fundo, BR 285 Rd., Km 292.7, Passo Fundo, Rio Grande do Sul, Brazil.
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Pastier C, De Hingh IHJT, Goéré D. New insights in the management of pseudomyxoma peritonei. J Surg Oncol 2024; 130:1316-1325. [PMID: 39206531 PMCID: PMC11826005 DOI: 10.1002/jso.27842] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2024] [Revised: 08/05/2024] [Accepted: 08/09/2024] [Indexed: 09/04/2024]
Abstract
While a rare entity, peritoneal pseudomyxoma treatment evolves. Decision-making criteria improve with imaging development and exploratory laparoscopy. Surgery remains at the core of the therapeutic strategy whatever disease progression. Complete cytoreduction plus hyperthermic intraperitoneal chemotherapy (HIPEC) is standard of care. Iterative cytoreduction or debulking is sometimes justified. Intraperitoneal chemotherapy modalities change with early postoperative HIPEC or pressurized intraperitoneal aerosol chemotherapy. Systemic or local treatment such as new chemo/immuno-therapies or BromAc should improve outcomes. Expertise and multicentric cooperation are more than ever needed.
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Affiliation(s)
- Clément Pastier
- Department of Digestive and Endocrine Surgery, Saint‐Louis Hospital AP‐HPUniversité Paris CitéParisFrance
| | | | - Diane Goéré
- Department of Digestive and Endocrine Surgery, Saint‐Louis Hospital AP‐HPUniversité Paris CitéParisFrance
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He L, Chen W. Recurrence and metastasis of pseudomyxoma peritonei: A rare case report. Radiol Case Rep 2024; 19:2929-2933. [PMID: 38745977 PMCID: PMC11091453 DOI: 10.1016/j.radcr.2024.04.034] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2024] [Revised: 04/07/2024] [Accepted: 04/09/2024] [Indexed: 05/16/2024] Open
Abstract
Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific clinical manifestations, leading to a high rate of misdiagnosis. This report describes the case of a 62-year-old male patient with recurrent and metastatic PMP. The patient first experienced unexplained epigastric pain and paroxysmal abdominal pain accompanied by distension over 8 years ago. He underwent surgical interventions for the condition in other hospitals in 2015 and 2018, respectively.
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Affiliation(s)
- Linjuan He
- Department of Radiology, Hainan General Hospital (Hainan Affiliated Hospital of Hainan Medical University), NO. 19, Xiuhua St, Xiuying Dic, Haikou, Hainan 570311, PR China
| | - Wangsheng Chen
- Department of Radiology, Hainan General Hospital (Hainan Affiliated Hospital of Hainan Medical University), NO. 19, Xiuhua St, Xiuying Dic, Haikou, Hainan 570311, PR China
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7
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Yano H, Gohda Y, Moran BJ, Suda R, Kokudo N. Long-term outcomes and survival analysis of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy in patients with pseudomyxoma peritonei at a newly established peritoneal malignancy centre in Japan. Ann Gastroenterol Surg 2024; 8:701-710. [PMID: 38957568 PMCID: PMC11216784 DOI: 10.1002/ags3.12791] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2023] [Revised: 12/20/2023] [Accepted: 02/26/2024] [Indexed: 07/04/2024] Open
Abstract
Background Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is established in the management of pseudomyxoma peritonei (PMP), selected cases of peritoneal mesothelioma, and resectable colorectal or ovarian peritoneal metastases in Western countries. However, the efficacy and feasibility of these techniques are not well established in the Asian population, and little has been reported on long-term survival outcomes for surgically resected PMP patients. Materials and Methods Retrospective analysis of a prospective database of short- and longer-term outcomes of consecutive patients who underwent CRS and HIPEC for PMP in a newly established peritoneal malignancy unit in Japan between 2010 and 2016. Results A total of 105 patients underwent CRS and HIPEC and 57 maximal tumor debulking (MTD) for pseudomyxoma peritonei. In the CRS group, the primary tumor was appendiceal in 94 patients (90%) followed by ovarian and colorectal. Major postoperative complications occurred in 22/105 patients (21%) with one in-hospital mortality (0.9%). The 5-year overall and disease-free survival rates for the CRS group were 74.2% and 50.1%, respectively. Multivariate analysis revealed unfavorable histology to be the significant predictor of reduced overall and disease-free survival. Completeness of cytoreduction, CA19-9, and CA125 were also associated with disease-free survival. Conclusions This is the first report on long-term outcomes and survival analysis of CRS and HIPEC for PMP in the Asian population. CRS and HIPEC can be conducted with reasonable safety and favorable survival in a new center. Complete tumor removal and histological type are the strongest prognostic factors for both overall and disease-free survival.
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Affiliation(s)
- Hideaki Yano
- Department of SurgeryNational Center for Global Health and MedicineTokyoJapan
- Division of Colorectal SurgeryUniversity Hospital SouthamptonSouthamptonUK
| | - Yoshimasa Gohda
- Department of SurgeryNational Center for Global Health and MedicineTokyoJapan
| | - Brendan J. Moran
- Peritoneal Malignancy Institute, North Hampshire HospitalBasingstokeUK
| | | | - Norihiro Kokudo
- Department of SurgeryNational Center for Global Health and MedicineTokyoJapan
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Awad A, Awad M, Alami M, Sablan AA, Shrateh ON, Jubran F. Successful treatment of pseudomyxoma peritonei (PMP) through cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC): A case report and literature review. Int J Surg Case Rep 2024; 119:109656. [PMID: 38677258 PMCID: PMC11067356 DOI: 10.1016/j.ijscr.2024.109656] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2024] [Revised: 04/11/2024] [Accepted: 04/19/2024] [Indexed: 04/29/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most commonly arising in the appendix. CASE PRESENTATION A 63-year-old male patient presented to the hospital with complaints of abdominal pain, significant weight loss, a palpable mass in the right iliac fossa, and evidence of ascites. A diagnosis of PMP was proposed based on findings from a computed tomography (CT) scan, which was subsequently confirmed through histopathological examination of a biopsy. The patient underwent successful treatment with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS-HIPEC). CLINICAL DISCUSSION Diagnosing PMP presents challenges due to its rarity and the potential for extensive spread throughout the peritoneal cavity, necessitating a multidisciplinary approach for successful treatment. CONCLUSION Pseudomyxoma peritonei is a rare yet medically significant condition. Documenting a case of this ailment in Palestine has the potential to advance medical understanding, raise awareness, and improve patient care standards within the local healthcare system. By documenting this uncommon condition, healthcare practitioners in Palestine can gain valuable insights into its manifestations, diagnostic processes, and treatment modalities. This contribution not only enriches the global medical literature but also promotes collaboration in addressing the challenges associated with rare diseases.
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Affiliation(s)
- Areej Awad
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | - Mahmoud Awad
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | - Muayyad Alami
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | | | - Oadi N Shrateh
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
| | - Fahmi Jubran
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine; Departement of Geneal Surgery, Al-Ahli Hospital, Hebron, Palestine
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Fujimoto D, Yonemura Y, Taniguchi K, Kobayashi H. Feasibility of Robot-Assisted Cytoreductive Surgery With Upper-Abdominal Peritonectomy for Pseudomyxoma Peritonei With Low Peritoneal Carcinomatosis Index: A Pilot Study. Surg Laparosc Endosc Percutan Tech 2024; 34:196-200. [PMID: 38417107 DOI: 10.1097/sle.0000000000001267] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2023] [Accepted: 01/18/2024] [Indexed: 03/01/2024]
Abstract
INTRODUCTION Our study's objective was to provide the method for, and preliminary findings from, robot-assisted cytoreductive surgery (r-CRS) combined with upper-abdominal peritonectomy in pseudomyxoma peritonei (PMP) with limited peritoneal surface malignancy (PSM). MATERIALS AND METHODS We conducted a retrospective pilot study on consecutive patients with PSM secondary to pseudomyxoma peritonei with a peritoneal cancer index (PCI) of under 10 who were indicated for r-CRS combined with upper-abdominal peritectomy. Perioperative and 30-day major morbidity and mortality characteristics were analyzed and compared with cases in which laparoscopic CRS (l-CRS) was performed under the same conditions. RESULTS Six patients underwent r-CRS combined with an upper-abdominal partial peritonectomy. Their mean PCI was 4.83. Complete cytoreduction was achieved in all patients. The mean duration of the operation was 156.8 minutes. There was no major complication and no mortality. The mean length of hospital stay after surgery was 6.33 days. r-CRS resulted in less blood loss, a lower C-reactive protein level, and a shorter length of hospital stay that were significantly different compared with those of l-CRS. CONCLUSIONS This is the initial technical report of a robotic approach for CRS combined with upper-abdominal peritonectomy. r-CRS with a combined upper-abdominal peritonectomy was shown to be safe and feasible for PMP with limited PSM.
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Affiliation(s)
| | - Yutaka Yonemura
- Department of Regional Cancer Therapy, Peritoneal Dissemination Center, Kishiwada Tokushukai Hospital, Kishiwada-city, Japan
| | - Keizo Taniguchi
- Department of Surgery, Teikyo University Hospital, Mizonokuchi
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Noguchi R, Yoshimatsu Y, Sin Y, Ono T, Tsuchiya R, Yoshida H, Kiyono T, Yonemura Y, Kondo T. Establishment and characterization of NCC-PMP2-C1: a novel patient-derived cell line of pseudomyxoma peritonei with signet ring cells. Hum Cell 2024; 37:511-522. [PMID: 38143259 DOI: 10.1007/s13577-023-01015-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/04/2022] [Accepted: 11/23/2023] [Indexed: 12/26/2023]
Abstract
Pseudomyxoma peritonei (PMP) is a rare phenomenon, characterized by accumulation of mucus in the abdominal cavity due to a mucinous neoplasm. Histologically, PMP is divided into three prognostic classes, namely low-grade mucinous carcinoma peritonei (LGMCP), high-grade mucinous carcinoma peritonei (HGMCP), and high-grade mucinous carcinoma peritonei with signet ring cells (HGMCP-S); HGMCP-S exhibits the worst prognosis. Complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy have been established as the standard therapy for PMP. However, 50% of patients with PMP experience a recurrence, and 30-40% are unable to receive the standard treatment due to invasive diseases. Therefore, novel therapies are required for their treatment. Although patient-derived cell lines are important tools for basic and pre-clinical research, PMP cell lines derived from patients with HGMCP-S have never been reported. Thus, we established a novel PMP cell line NCC-PMP2-C1, using surgically resected tumor tissue from a patient with HGMCP-S. NCC-PMP2-C1 cells were maintained for more than five months and passaged 30 times under culture conditions. NCC-PMP2-C1 cells exhibited multiple deletions and somatic mutations, slow growth, histological features, and dissemination of tumor cells in nude mice. Screening for the anti-proliferative effects of anti-cancer drugs on cells revealed that bortezomib, mubritinib, and romidepsin had a significant response against NCC-PMP2-C1 cells. Thus, the NCC-PMP2-C1 cell line is the first PMP cell line harboring signet ring cells and will be a valuable resource for basic and preclinical studies of HGMCP-S.
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Affiliation(s)
- Rei Noguchi
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan
| | - Yuki Yoshimatsu
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan
| | - Yooksil Sin
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan
| | - Takuya Ono
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan
| | - Ryuto Tsuchiya
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan
| | - Hiroshi Yoshida
- Department of Diagnostic Pathology, National Cancer Center Hospital, Tokyo, Japan
| | - Tohru Kiyono
- Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa, Japan
| | - Yutaka Yonemura
- NPO to Support Peritoneal Surface Malignancy Treatment, Japanese/Asian School of Peritoneal Surface Oncology, Kyoto, Japan
- Department of Regional Cancer Therapy, Peritoneal Surface Malignancy Center, Kishiwada Tokushukai Hospital, Kishiwada, Japan
- Department of Regional Cancer Therapy, Peritoneal Surface Malignancy Center, Kusatsu General Hospital, Shiga, Japan
| | - Tadashi Kondo
- Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan.
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Aso K, Gohda Y, Hotta M, Minamimoto R, Shimizu Y, Uemura Y, Yano H. Clinical Effectiveness of Preoperative 18F-FDG PET/CT in Predicting Pathological Tumor Grade in Patients with Pseudomyxoma Peritonei Originating from Appendix: A Retrospective Cohort Study. Ann Surg Oncol 2024; 31:1990-1995. [PMID: 38082170 DOI: 10.1245/s10434-023-14755-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2023] [Accepted: 11/25/2023] [Indexed: 02/08/2024]
Abstract
BACKGROUND Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the standard treatment for patients with pseudomyxoma peritonei (PMP). In some malignancies, the standard uptake value of positron emission tomography with 2-deoxy-2-18F-fluoro-D-glucose integrated with computed tomography (18F-FDG PET/CT) is now accepted as a reliable indicator of neoplastic behavior. This study aimed to evaluate the association between the maximum standardized uptake value (SUVmax) and pathological grade in patients with PMP and to investigate the significance of SUVmax in the preoperative assessment of these patients. PATIENTS AND METHODS In this retrospective single-center study, consecutively enrolled patients diagnosed with PMP of appendiceal origin underwent preoperative 18F-FDG PET/CT. SUVmax was calculated as the highest SUVmax value in the abdomen excluding the primary site. SUVmax was compared with the pathological grade (low or high grade) of PMP tumors according to the World Health Organization classification and further analyzed with respect to the estimated cutoff point, sensitivity, specificity, and receiver operating characteristic. RESULTS In total, 160 patients were included. CRS was successfully performed in 93 patients and palliative debulking surgery in 67 patients. The pathological grade was high in 45 patients and low in 115. High-grade patients had a higher median SUVmax on 18F-FDG PET/CT than did low-grade patients (3.83 versus 2.34, p < 0.001). The highest area under the curve was 0.81, with a sensitivity of 77.8%, specificity of 72.3%, and cutoff point of 2.63. CONCLUSION This study suggests that the SUVmax of preoperative 18F-FDG PET/CT is associated with the pathological grade in patients with PMP.
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Affiliation(s)
- Kenta Aso
- Department of Surgery, National Center for Global Health and Medicine, Tokyo, Japan
| | - Yoshimasa Gohda
- Department of Surgery, National Center for Global Health and Medicine, Tokyo, Japan.
| | - Masatoshi Hotta
- Division of Nuclear Medicine, Department of Radiology, National Center for Global Health and Medicine, Tokyo, Japan
| | - Ryogo Minamimoto
- Division of Nuclear Medicine, Department of Radiology, National Center for Global Health and Medicine, Tokyo, Japan
| | - Yosuke Shimizu
- Center of Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan
| | - Yukari Uemura
- Center of Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan
| | - Hideaki Yano
- Consultant Colorectal Surgeon, University Hospital Southampton NHS Foundation Trust, Southampton, UK
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Yoshizaki Y, Gohda Y, Inagaki F, Kataoka A, Takemura N, Miyazaki H, Igari T, Kiyomatsu T, Yano H, Kokudo N. A case of pseudomyxoma peritonei arising from a perforated intraductal papillary mucinous neoplasm that underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Clin J Gastroenterol 2024; 17:188-197. [PMID: 37980306 DOI: 10.1007/s12328-023-01890-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/23/2023] [Accepted: 10/25/2023] [Indexed: 11/20/2023]
Abstract
Pseudomyxoma peritonei (PMP) of pancreatic origin arising from an intraductal papillary mucinous neoplasm (IPMN) is rare. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has been established as the optimal treatment for PMP. However, the benefits and safety of CRS with HIPEC for treating PMP of pancreatic origin remain unclear. Herein, we describe a case of PMP of pancreatic origin that was treated with CRS and HIPEC without postoperative complications. A 75-year-old woman was referred to our department. Computed tomography (CT) revealed a multilocular cystic tumor in the pancreatic tail, notable mucinous ascites in the abdominal cavity, and scalloping of the liver and spleen. CT did not reveal the appendix, and the ovaries were normal in size. The patient was diagnosed with PMP of pancreatic origin, and CRS and HIPEC were performed. Intraoperatively, the pancreatic tumor was perforated, and there was a large amount of mucinous ascites. We performed distal pancreatectomy in addition to CRS and HIPEC, with no intraoperative complications. The postoperative course was uneventful, and the patient survived after 6 months without recurrence. CRS with HIPEC may be a feasible treatment option for PMP of pancreatic origin.
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Affiliation(s)
- Yuhi Yoshizaki
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Yoshimasa Gohda
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan.
| | - Fuyuki Inagaki
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Atsuko Kataoka
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Nobuyuki Takemura
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Hideki Miyazaki
- Department of Pathology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Toru Igari
- Department of Pathology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Tomomichi Kiyomatsu
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Hideaki Yano
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
| | - Norihiro Kokudo
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-Ku, Tokyo, 162-8655, Japan
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13
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Wang B, Ma R, Shi G, Fan X, Rao B, Xu H. Hyperthermic intraperitoneal chemotherapy in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei: a 10-year treatment experience in China. Orphanet J Rare Dis 2024; 19:8. [PMID: 38178189 PMCID: PMC10768245 DOI: 10.1186/s13023-023-02995-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2022] [Accepted: 12/13/2023] [Indexed: 01/06/2024] Open
Abstract
BACKGROUND To explore the application value of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with incomplete cytoreduction for appendiceal pseudomyxoma peritonei (PMP). METHODS We retrospectively analyzed the clinical data of 526 patients with incomplete cytoreduction for appendiceal PMP to discover its prognostic factors, and the therapeutic value of HIPEC. RESULTS The 5-year and 10-year overall survival rates of patients after cytoreductive surgery (CRS) treated with HIPEC were significantly higher than those without HIPEC (5y-OS: 58% vs. 48%, 10y-OS: 37% vs. 16%, P = 0.032). The median progression-free survival (PFS) following CRS was 20 months, with a 20% 3-year PFS. The median PFS following CRS + HIPEC was 33 months, with a 60% 3-year PFS (P = 0.000). Univariate analysis indicated that HIPEC, gender, completeness of cytoreduction (CCR) and pathological grade had statistical difference. Multivariate analysis showed that CRS without HIPEC and high pathological grade were independent risk factors for poor prognosis and rapid tumor progression. CONCLUSIONS HIPEC may prolong the survival in patients with incomplete cytoreduction for low-grade appendiceal PMP. High pathological grade indicates poor survival and rapid tumor progression.
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Affiliation(s)
- Bing Wang
- Department of Myxoma, Aerospace Center Hospital, Beijing, 100049, China
- Department of Gastrointestinal Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, 100038, China
| | - Ruiqing Ma
- Department of Myxoma, Aerospace Center Hospital, Beijing, 100049, China
| | - Guanjun Shi
- Department of Myxoma, Aerospace Center Hospital, Beijing, 100049, China
| | - Xiwen Fan
- Department of Myxoma, Aerospace Center Hospital, Beijing, 100049, China
| | - Benqiang Rao
- Department of Gastrointestinal Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, 100038, China.
| | - Hongbin Xu
- Department of Myxoma, Aerospace Center Hospital, Beijing, 100049, China.
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14
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Kamada Y, Hida K, Yonemura Y, Nakakura A, Kitai T, Mizumoto A, Yoshida S, Tokoro Y, Obama K. Analysis of the characteristics and outcomes of patients with pseudomyxoma peritonei of appendiceal origin treated with curative-intent surgery. Surg Oncol 2023; 51:102012. [PMID: 37972508 DOI: 10.1016/j.suronc.2023.102012] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2023] [Revised: 10/07/2023] [Accepted: 10/22/2023] [Indexed: 11/19/2023]
Abstract
BACKGROUND There are no previous studies on pseudomyxoma peritonei regarding the details of surgical procedures included in cytoreductive surgery and quantitative evaluation for peritoneal metastases by region in the abdominal cavity. This study aimed to describe the characteristics and procedural details involved in cytoreductive surgery, and survival outcomes of patients with pseudomyxoma peritonei originating from appendiceal mucinous neoplasm, and identify differences in the difficulty of cytoreductive surgery based on tumor location. METHODS Patient characteristics and survival outcomes were studied through a retrospective review. The complete cytoreduction rate (i), the 5-year survival rate for patients with complete cytoreduction (ii), and an index as a complement (i × ii × 100) were described for patients who had tumors larger than 50 mm in one of the 13 regions of the abdominal cavity. RESULTS A total of 989 patients were treated with curative-intent cytoreductive surgery. The median peritoneal cancer index was 18 (interquartile range, 6-29), with complete cytoreduction achieved in 702 patients (71%); the major complication rate was 17%. The median overall survival was 92.9 months, compared to 53.8 months for patients who underwent total gastrectomy and 30.4 months for those who underwent total colectomy. In the 13 abdominal regions, the index scores indicating cytoreduction difficulty were categorized into three risk groups: upper and mid-abdominal (>20), lateral abdominal (10-20), and small bowel (<10). CONCLUSIONS Cytoreductive surgery offered favorable survival outcomes, even in cases involving total gastrectomy. The difficulty of achieving complete cytoreduction varied across abdominal regions and was classified into three levels.
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Affiliation(s)
- Yasuyuki Kamada
- Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan; Department of Regional Cancer Therapies, Peritoneal Surface Malignancy Treatment Center, Kishiwada Tokushukai Hospital, Kishiwada City, Osaka, Japan
| | - Koya Hida
- Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
| | - Yutaka Yonemura
- Department of Regional Cancer Therapies, Peritoneal Surface Malignancy Treatment Center, Kishiwada Tokushukai Hospital, Kishiwada City, Osaka, Japan; Department of Peritoneal Surface Malignancy Treatment, Omi Medical Center, Kusatsu City, Shiga, Japan
| | - Akiyoshi Nakakura
- Department of Biomedical Statistics and Bioinformatics, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Toshiyuki Kitai
- Department of Regional Cancer Therapies, Peritoneal Surface Malignancy Treatment Center, Kishiwada Tokushukai Hospital, Kishiwada City, Osaka, Japan
| | - Akiyoshi Mizumoto
- Department of Peritoneal Surface Malignancy Treatment, Omi Medical Center, Kusatsu City, Shiga, Japan
| | - Shinya Yoshida
- Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Yukinari Tokoro
- Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Kazutaka Obama
- Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
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15
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Kusamura S, Busico A, Conca E, Capone I, Agnelli L, Lorenzini D, Brich S, Angelini M, Volpi CC, Trupia DV, Lagano V, Torelli T, Gloghini A, Baratti D, Guaglio M, Milione M, Deraco M, Perrone F. A2AR Expression and Immunosuppressive Environment Independent of KRAS and GNAS Mutations in Pseudomyxoma Peritonei. Biomedicines 2023; 11:2049. [PMID: 37509688 PMCID: PMC10377259 DOI: 10.3390/biomedicines11072049] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2023] [Revised: 07/07/2023] [Accepted: 07/18/2023] [Indexed: 07/30/2023] Open
Abstract
In pseudomyxoma peritonei (PMP), KRAS and GNAS mutations are frequent. We hypothesized that these mutations may contribute to the suppression of antitumor immunity: KRAS may induce GMCSF expression, while GNAS may enhance the expression of cyclic adenosine monophosphate and A2AR signaling. This study aimed to explore possible mechanisms facilitated by KRAS and GNAS mutations for escaping immune surveillance. Additionally, we looked for new potential therapeutic and prognostic targets in this rare disease which is poorly characterized at the molecular level. GM-CSF, A2AR, CD73, CD39, and PD-L1 expression was investigated by immunohistochemistry in 40 PMPs characterized for GNAS and KRAS mutational status. Immune cell populations were studied by immunohistochemistry and nanostring nCounter®. Following the criteria of a prognostic nomogram reported for PMP, we stratified the patients into two different risk groups, with 28 "low-risk" and 12 "high-risk" patients. We observed the expression of GM-CSF (74%); CD39 (37%); CD73 (53%); A2AR (74%); and PD-L1 (16%) which was unrelated to GNAS or KRAS status. The tumor microenvironment showed the presence of CD4+ T cells (86%); CD8+ T cells (27%); CD20+ B (67%); CD15+ cells (86%); and CD163+ M2 macrophages (67%), while CD56+ NK cells were absent. CD163 expression (27%) in PMP tumor cells was associated with poor prognosis. GNAS mutation and A2AR expression were not associated with a specific immune transcriptional signature. However, the expression assay revealed 21 genes associated with prognosis. The "high-risk" patients exhibited worse progression-free survival (HR = 2.3, CI 95%: 1.1-5.1, p = 0.034) and significant downregulation of MET, IL8, PPARG, DTX4, HMGA1, ZIC2, WNT5B, and CCRL2. In conclusion, we documented the presence of immunosuppressive factors such as GM-CSF, A2AR, and PD-L1 in PMP. These factors were not associated with GNAS and KRAS status and could be explored as therapeutic molecular targets. Additionally, a set of potential prognostic biomarkers, including CD163 expression in tumor cells, deserve further investigation.
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Affiliation(s)
- Shigeki Kusamura
- Peritoneal Surface Malignancy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Adele Busico
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Elena Conca
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Iolanda Capone
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Luca Agnelli
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
- Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Daniele Lorenzini
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Silvia Brich
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Marta Angelini
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Chiara Costanza Volpi
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Desirè Viola Trupia
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Vincenzo Lagano
- 1st Pathology Division, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Tommaso Torelli
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Annunziata Gloghini
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Dario Baratti
- Peritoneal Surface Malignancy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Marcello Guaglio
- Peritoneal Surface Malignancy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Massimo Milione
- 1st Pathology Division, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Marcello Deraco
- Peritoneal Surface Malignancy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
| | - Federica Perrone
- Laboratory of Diagnostic and Molecular Research, Department of Diagnostic Innovation, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy
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16
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González Bayón L, Martín Román L, Lominchar PL. Appendiceal Mucinous Neoplasms: From Clinic to Pathology and Prognosis. Cancers (Basel) 2023; 15:3426. [PMID: 37444536 DOI: 10.3390/cancers15133426] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2023] [Revised: 06/20/2023] [Accepted: 06/26/2023] [Indexed: 07/15/2023] Open
Abstract
Appendiceal mucinous neoplasms have been classified differently over time causing confusion when comparing results between working groups in this field and establishing a prognosis of the disease. A historical perspective of the different classification systems of these tumors is essential for the understanding of the evolution of concepts and histopathological definitions that have led up to the present moment. We carried out a systematic review of the pathological classifications of appendiceal mucinous tumors and how they have included the new criteria resulting from clinical and pathological research. The latest classifications by PSOGI and AJCC 8th edition Cancer Staging have made a great effort to incorporate the new pathological descriptions and develop prognostic groups. The introduction of these new classification systems has posed the challenge of verifying how they adapt to our casuistry and which one defines best the prognosis of our patients. We reclassified our series of patients treated for mucinous appendiceal tumors with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy following the PSOGI and the AJCC 8th edition criteria and concluded that both classifications correspond well with the OS and DFS of these patients, with some advantage relative to the PSOGI classification due to a better histopathological description of the different groups.
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Affiliation(s)
- Luis González Bayón
- Peritoneal Carcinomatosis Unit, Department of General Surgery, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
- Faculty of Medicine, Universidad Complutense de Madrid, 28040 Madrid, Spain
| | - Lorena Martín Román
- Peritoneal Carcinomatosis Unit, Department of General Surgery, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
- Faculty of Medicine, Universidad Complutense de Madrid, 28040 Madrid, Spain
| | - Pablo Lozano Lominchar
- Peritoneal Carcinomatosis Unit, Department of General Surgery, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
- Faculty of Medicine, Universidad Complutense de Madrid, 28040 Madrid, Spain
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17
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Chandrakumaran K, Carr NJ, Mohamed F, Cecil TD, Moran BJ. Development and Validation of Nomograms to Predict Survival in Patients Undergoing Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendiceal Origin. JAMA Surg 2023; 158:522-530. [PMID: 36920381 PMCID: PMC10018403 DOI: 10.1001/jamasurg.2023.0112] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2022] [Accepted: 11/05/2022] [Indexed: 03/16/2023]
Abstract
Importance Pseudomyxoma peritoni, a rare condition characterized by mucinous ascites and peritoneal deposits, mainly originates from a ruptured mucinous appendix tumor and is considered an indolent disease but can progress and become fatal. Optimal treatment to improve cure and survival rates involves complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Accurate predictive models are useful in supporting and informing treatment strategies and stratifying patient follow-up. Objective To evaluate the prognostic significance of clinically important variables and generate validated nomograms to predict overall (OS) and disease-free survival (DFS) following CCRS and hyperthermic intraperitoneal HIPEC for pseudomyxoma peritonei (PMP) of appendiceal origin. Design, Setting, and Participants This retrospective study used prospectively collected data on patients who had cytoreductive surgery (CRS) and HIPEC in a single institution between 1994 and 2018. The cohort was randomly allocated into development (70%) and validation (30%) sets. Univariate and multivariate analyses were performed with Cox proportional hazards regression. Main Outcomes and Measures A prediction model was developed with significant prognostic factors identified by multivariate analysis. The model's prognostic performance was evaluated with the concordance index (C index). The nomogram was calibrated by comparing the predicted and observed probabilities. Results Of 2637 CRS and HIPEC operations, 1102 patients (female, 64.4%; median age [IQR], 57.0 [48.0-66.0] years) (41.8%) had CCRS for PMP of appendiceal origin. Elevated tumor markers, peritoneal carcinomatosis index, gastrectomy, and tumor grade were independent predictive factors for DFS. Gender, age, elevated tumor makers, peritoneal carcinomatosis index, and tumor grade influenced OS. The nomograms were generated with respective prognostic factors. The nomograms showed good performance in predicting survival. Median OS of the cohort was 16.5 years (95% CI, 13.7-19.2) with a 5-year probability of survival of 80.2%. The median DFS was 10.3 years (95% CI, 7.2- 13.3) and the 5-year probability of recurrence-free survival was 60.5%. Conclusions and Relevance Clinically important independent predictors for survival and recurrence were selected to develop the nomograms for OS and DFS. These 2 nomograms are user friendly and useful tools for patient management with clinical trial design applications.
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Affiliation(s)
- Kandiah Chandrakumaran
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom
| | - Norman John Carr
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom
| | - Faheez Mohamed
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom
| | - Thomas Desmond Cecil
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom
| | - Brendan John Moran
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, United Kingdom
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18
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Hossain S, Manna S, De U. The Giant “Belly” Full of “Jelly”. Indian J Surg 2023. [DOI: 10.1007/s12262-023-03730-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/17/2023] Open
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19
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Borg P, Ng HHL, Mullan D, Aziz O, Laasch HU. Ultrasound-guided day-case wide-bore percutaneous mucin aspiration in advanced pseudomyxoma peritonei. Clin Radiol 2023; 78:e458-e462. [PMID: 36842914 DOI: 10.1016/j.crad.2023.01.016] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Revised: 01/26/2023] [Accepted: 01/26/2023] [Indexed: 02/18/2023]
Abstract
AIM To evaluate percutaneous ultrasound-guided day-case mucin aspiration in advanced pseudomyxoma peritonei (PMP) using a wide-bore drain with regards to its safety and efficacy. MATERIALS AND METHODS All patients who underwent percutaneous mucin aspiration for PMP between 2019-2021 at a single national peritoneal tumour service were included in this study. Under local anaesthesia, a suction-enabled 28-32 F catheter was used for drainage following wire-guided track dilatation. The volume drained and difference in abdominal girth pre- and post-procedure were measured. Patients graded difficulty in breathing and abdominal discomfort pre- and post-procedure. Histology reports were reviewed. RESULTS Sixteen patients received 56 percutaneous mucin aspirations between 2019-2021. The aetiology was a low-grade appendiceal mucinous neoplasm (LAMN) in 50% of patients. The mean amount of mucin drained was 7,320 ± 3,000ml (range 300-13,500 ml). The mean reduction in abdominal girth post-procedure was 12.2 ± 5 cm (range 0-27 cm). Only grade 1 complications were observed. CONCLUSION Percutaneous ultrasound-guided day-case aspiration of mucin for advanced and recurrent PMP using a wide-bore drain is a safe and effective procedure. It may be used in the palliative setting or as a bridge to surgery in the very symptomatic patient or if there is a reversible contraindication to surgery.
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Affiliation(s)
- P Borg
- Department of Radiology, The Christie NHS Foundation Trust, Manchester, UK.
| | - H H-L Ng
- School of Medicine, Faculty of Medicine and Health, University of Leeds, Leeds, UK
| | - D Mullan
- Department of Radiology, The Christie NHS Foundation Trust, Manchester, UK
| | - O Aziz
- Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester, UK; Faculty, Institute of Cancer Sciences, University of Manchester, Manchester, UK
| | - H-U Laasch
- Department of Radiology, The Christie NHS Foundation Trust, Manchester, UK; Department of Natural Sciences, University of Chester, Chester, UK
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20
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Brimo Alsaman MZ, Anbar A, Nawlo A, Almooay A, Darwish A, Mohammad M. Incidental diagnosis of pseudomyxoma peritonei by laparoscopy: a rare case from Syria. J Surg Case Rep 2022; 2022:rjac558. [PMID: 36518651 PMCID: PMC9731612 DOI: 10.1093/jscr/rjac558] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2022] [Accepted: 11/19/2022] [Indexed: 12/14/2022] Open
Abstract
Pseudomyxoma peritonei (PMP) is a rare malignancy of the abdomen, which is usually described as a gelatinous peritoneal fluid. A 33-year-old man came to the ER complaining of recurring abdominal pain with abdominal distention for the past 3 months. Abdominal ultrasound revealed moderate amount of turbulent ascitic fluid with septations, in addition to a mass with irregular margins consisting of liquid and cysts. Abdomen and pelvis computed tomography scan showed free abdominal fluid. A decision was made for abdominal diagnostic laparoscopy with biopsies from the peritoneum, mesenteries and the gelatinous fluid. We could not investigate all the abdominal cavities and appendix due to the presence of severe adhesions and tuberculosis suspension. The pathology report indicated PMP. The patient was referred to an oncologist for chemotherapy consultation. The diagnosis can be challenging, as the symptoms and signs vary from patient to another; most cases may be asymptomatic and discovered incidentally during laparoscopy.
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Affiliation(s)
| | - Ahmad Anbar
- Faculty of Medicine, University of Aleppo, Aleppo 15310, Syria
| | - Ahmad Nawlo
- General Surgery Department, Aleppo University Hospital, Aleppo 15310, Syria
| | - Adeeb Almooay
- General Surgery Department, Tishreen Hospital, Damascus, Syria
| | - Ahmad Darwish
- Pathology Department, Tishreen Hospital, Damascus, Syria
| | - Mazen Mohammad
- General Surgery Department, Abd Al Wahab Agha Hospital, Aleppo, Syria
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21
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Gao X, Kou L, Guan H, Tian H, Jia J, Bai Y, Bai W, Di Y, Ma R, Wang X. Transfusion of stored autologous blood in patients with low-grade pseudomyxoma peritonei: A retrospective analysis of its safety and outcome. Front Oncol 2022; 12:1022426. [PMID: 36276137 PMCID: PMC9584637 DOI: 10.3389/fonc.2022.1022426] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Accepted: 09/20/2022] [Indexed: 11/16/2022] Open
Abstract
BACKGROUND Pseudomyxoma peritonei is a rare disease that presents as a malignant tumor on the peritoneal surface. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the standard treatment for this disease and frequently requires a red blood cell transfusion. However, due to the limited collection and supply of allogeneic blood, surgical treatment may be delayed due to inadequate preparation of allogeneic blood in the course of clinical treatment. This study aimed to evaluate the safety and efficacy of transfusion of stored autologous blood in patients with low-grade pseudomyxoma peritonei. METHODS Pseudomyxoma peritonei patients who received cytoreductive surgery combined with heat-infused peritoneal chemotherapy were divided into two groups: transfusion of allogeneic blood and transfusion of stored autologous blood. A comparison of the differences in multiple factors between the two groups was performed, including tumor recurrence, survival time, hemoglobin and hematocrit levels, coagulation function (prothrombin time, activated partial thromboplastin time, and fibrinogen), total hospital stay duration, and incidence of serious adverse events after surgery. RESULTS Propensity scores matching analysis yielded 34 patients with allogeneic blood transfusion and 34 patients with stored autologous blood transfusion. Comparison analysis did not show statistical differences in several factors, including age, tumor grade, tumor recurrence rate after surgery, etc., between the two groups. The cytoreductive degree was considered an independent risk factor for tumor recurrence. The pseudomyxoma peritonei patients in the autologous transfusion group had a higher 5-year survival rate and a longer survival time. Moreover, transfusion of stored autologous blood did not increase the rate of tumor recurrence, or the total hospital stay duration after surgery, the hemoglobin level and coagulation function were well stabilized within 24 h after surgery, and there was a low incidence of serious adverse events. CONCLUSION The clinical application of transfusion of stored autologous blood in pseudomyxoma peritonei patients is safe and effective.
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Affiliation(s)
- Xiaoyun Gao
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Liduo Kou
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Hang Guan
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Hua Tian
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Junhui Jia
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Yu Bai
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Wei Bai
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Yanhui Di
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
| | - Ruiqing Ma
- Department of Myxoma, Aerospace Center Hospital, Beijing, China
| | - Xinhua Wang
- Department of Blood Transfusion, Aerospace Center Hospital, Beijing, China
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22
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Feng JN, Hu AJ, Xu L, Liu LL, Liu GZ, Wang JX, Gao P, Liu CR, Wang SF, Zhan SY. Prevalence and incidence of pseudomyxoma peritonei in urban China: A nationwide retrospective cohort study. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2022; 48:2226-2232. [PMID: 35764460 DOI: 10.1016/j.ejso.2022.06.013] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2022] [Revised: 06/03/2022] [Accepted: 06/08/2022] [Indexed: 12/09/2022]
Abstract
BACKGROUND Pseudomyxoma peritonei (PMP) is an extremely rare condition. Information regarding the disease burden of PMP in developing countries is limited. This study aimed to determine the epidemiology of PMP in China. METHODS PMP data were extracted from the national databases of Urban Basic Medical Insurance. All cases were identified using the International Classification of Diseases (ICD) codes and Chinese diagnostic terms. The national prevalence from 2012 to 2016 and incidence in 2016 were estimated. RESULTS In total, 153 patients with PMP were identified. The crude prevalence of PMP in 2016 was 2.47 (95% confidence interval [CI] 1.71 to 3.23) per million person-year, with a higher prevalence in females than males. Prevalence increased with age, with the first peak in those aged 15-29 years and the highest in those aged >80 years. The crude incidence of PMP in 2016 was 1.19 (95% CI 0.59 to 1.78) per million person-years. Similar to the prevalence, the rates were higher in women than in men. The incidence also increased with age, with the highest prevalence in those aged >80 years. Besides, the most frequent comorbidities before and after the first diagnosis of PMP were unspecified secondary malignancies and malignancies of unspecified sites, followed by abdominal malignant tumours. CONCLUSIONS The rate of PMP was lower in mainland China than in European countries and increased with advancing age. Women were more likely to have PMP than men. Furthermore, an insufficient understanding of this rare disease presents a major challenge in accurately evaluating the disease burden.
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Affiliation(s)
- Jing-Nan Feng
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China
| | - A-Jin Hu
- Department of Pathology, Peking University Third Hospital, Beijing, 100191, China
| | - Lu Xu
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China
| | - Li-Li Liu
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China
| | - Guo-Zhen Liu
- Peking University Health Information Technology Co. Ltd, Beijing, 100080, China
| | - Jin-Xi Wang
- Shanghai Songsheng Business Consulting Co. LTD, Beijing, 100000, China
| | - Pei Gao
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China
| | - Cong-Rong Liu
- Department of Pathology, Third Hospital, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, 100191, China.
| | - Sheng-Feng Wang
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China.
| | - Si-Yan Zhan
- Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing, 100191, China; Research Center of Clinical Epidemiology, Peking University Third Hospital, Beijing, 100191, China; Institute for Artificial Intelligence, Peking University, Beijing, 100091, China.
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Sarpietro G, Iraci Sareri M, Bonanno GM, Matarazzo MG, Cianci A. Incidental Diagnosis of Pseudomyxoma Peritonei: A Case Report. Cureus 2022; 14:e23425. [PMID: 35475088 PMCID: PMC9030654 DOI: 10.7759/cureus.23425] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/23/2022] [Indexed: 11/09/2022] Open
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24
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Ponzini F, Kowal L, Ghafoor M, Goldberg A, Chan J, Lamm R, Cannaday SM, Richard SD, Nevler A, Lavu H, Bowne WB, Rosenblum NG. Rare occurrence of pseudomyxoma peritonei (PMP) syndrome arising from a malignant transformed ovarian primary mature cystic teratoma treated by cytoreductive surgery and HIPEC: a case report. World J Surg Oncol 2022; 20:78. [PMID: 35272690 PMCID: PMC8915470 DOI: 10.1186/s12957-022-02548-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Accepted: 02/25/2022] [Indexed: 11/29/2022] Open
Abstract
Background Pseudomyxoma peritonei (PMP) syndrome is a disease process that typically occurs from ruptured appendiceal mucocele neoplasms. PMP syndrome arising from malignant transformation of an ovarian primary mature cystic teratoma (MCT) is a pathogenesis rarely encountered. Case Presentation Herein, we report a 28-year-old patient evaluated and treated for a right ovarian mass and large volume symptomatic abdominopelvic mucinous ascites. Molecular profiling and genetic analysis revealed mutations in ATM, GNAS, and KRAS proteins while IHC demonstrated gastrointestinal-specific staining for CK20, CDX2, CK7, and SATB2. Peritoneal cytology showed paucicellular mucin. Diffuse peritoneal adenomucinosis (DPAM) variant of PMP arising from a ruptured ovarian primary MCT after malignant transformation to a low-grade appendiceal-like mucinous neoplasm was ultimately confirmed. Treatment included staged therapeutic tumor debulking and right salpingo-oophorectomy followed by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Conclusions Our report builds upon the existing literature supporting this aggressive treatment option reserved for advanced abdominal malignancies utilized in this patient with a rare clinical entity.
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Affiliation(s)
- Francesca Ponzini
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA
| | - Luke Kowal
- Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA, USA
| | - Mariam Ghafoor
- Department of Pathology, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Allison Goldberg
- Department of Pathology, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Joanna Chan
- Department of Pathology, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Ryan Lamm
- Department of Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Shawnna M Cannaday
- Department of Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Scott D Richard
- Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Avinoam Nevler
- Department of Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Harish Lavu
- Department of Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA
| | - Wilbur B Bowne
- Department of Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
| | - Norman G Rosenblum
- Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
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25
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Taguchi A, Rokutan H, Oda K, Tanikawa M, Tanimoto S, Sone K, Mori M, Tsuruga T, Kohsaka S, Tatsuno K, Shinozaki-Ushiku A, Miyagawa K, Mano H, Aburatani H, Ushiku T, Osuga Y. Genetic diagnosis of pseudomyxoma peritonei originating from mucinous borderline tumor inside an ovarian teratoma. BMC Med Genomics 2022; 15:51. [PMID: 35255903 PMCID: PMC8900394 DOI: 10.1186/s12920-022-01188-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2021] [Accepted: 02/18/2022] [Indexed: 11/12/2022] Open
Abstract
Background Pseudomyxoma peritonei is a rare disease condition mainly caused by primary mucinous tumors from the appendix and rarely from the ovary, such as when mucinous ovarian tumors arise from within a teratoma. Molecular analyses of pseudomyxoma from the appendix showed that KRAS and GNAS pathogenic variants are common genetic features of pseudomyxoma peritonei. However, the origin of the tumors is difficult to be identified via genetic variants alone. This study presents a case of pseudomyxoma peritonei of ovarian origin, which was diagnosed by comprehensive genomic profiling with ploidy analysis in a series of primary, recurrent, and autopsy tumor specimens. Case presentation A 40-year-old woman was diagnosed with Stage IC2 mucinous ovarian tumor of borderline malignancy with mature cystic teratoma, upon clinical pathology. Immunohistochemical analysis suggested that the mucinous tumor was derived from the intestinal component of an ovarian teratoma. Three years later, intraperitoneal recurrence was detected, which subsequently progressed to pseudomyxoma peritonei. Genomic analysis detected KRAS (G12D), GNAS (R201C), and FBXW7 (R367*) variants in the primary tumor. In addition, the tumor showed aneuploidy with loss of heterozygosity (LOH) in all its chromosomes, which suggested that the primary ovarian tumor was derived from germ cells. Existence of one Barr body suggested the existence of uniparental disomy of the tumors throughout the genome, instead of a haploid genotype. All three pathogenic variants remained positive in the initial recurrent tumor, as well as in the paired DNA from the whole blood in pseudomyxoma peritonei. The pathogenic variant of KRAS (G12D) was also identified in the autopsy specimen of the appendix by droplet digital polymerase chain reaction. Conclusions This study pathologically and genetically confirmed that the primary ovarian borderline tumor was derived from the intestinal component of an ovarian teratoma, and that the subsequent pseudomyxoma peritonei progressed from the primary ovarian tumor. Integrative genomic analysis was useful to identify cellular origin of tumors, as well as to precisely interpret the process of disease progression. Supplementary Information The online version contains supplementary material available at 10.1186/s12920-022-01188-x.
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Affiliation(s)
- Ayumi Taguchi
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Hirofumi Rokutan
- Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Katsutoshi Oda
- Division of Integrative Genomics, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan.
| | - Michihiro Tanikawa
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Saki Tanimoto
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kenbun Sone
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Mayuyo Mori
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Tetsushi Tsuruga
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Shinji Kohsaka
- Division of Cellular Signaling, National Cancer Center Research Institute, Tokyo, Japan
| | - Kenji Tatsuno
- Genome Science Division, Research Center for Advanced Science and Technology, The University of Tokyo, Tokyo, Japan
| | - Aya Shinozaki-Ushiku
- Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kiyoshi Miyagawa
- Laboratory of Molecular Radiology, Center for Disease Biology and Integrative Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Hiroyuki Mano
- Division of Cellular Signaling, National Cancer Center Research Institute, Tokyo, Japan
| | - Hiroyuki Aburatani
- Genome Science Division, Research Center for Advanced Science and Technology, The University of Tokyo, Tokyo, Japan
| | - Tetsuo Ushiku
- Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yutaka Osuga
- Department of Obstetrics and Gynecology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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26
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Wei PK, Gupta M, Tsai LL, Lee KS, Jaramillo AM, Smith MP, LeGout JD, Shenoy-Bhangle AS. Spectrum of MRI Features of Mucin-producing Neoplasms in the Abdomen and Pelvis. Radiographics 2022; 42:469-486. [PMID: 35061517 DOI: 10.1148/rg.210055] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Mucin-producing neoplasms in the abdomen and pelvis are a distinct entity, separate from simple fluid-containing neoplasms and loculated fluid collections. Mucin is a thick gelatinous substance and-owing to its high water content-has imaging features that can be mistaken for those of simple fluid-containing neoplasms with multiple imaging modalities. However, mucin-producing neoplasms arise from specific organs in the abdomen and pelvis, with unique imaging appearances, knowledge of which is important to guide accurate diagnosis and management. With its large field of view and high soft-tissue resolution, MRI has advantages over other imaging modalities in characterizing these neoplasms. The authors focus on the spectrum of MRI features of such mucin-producing neoplasms and illustrate how-despite a varied organ origin-some of these neoplasms share similar MRI and histopathologic features, thereby helping narrow the differential diagnosis. One common finding in these tumors is that the presence of internal complexity and solid enhancing components increases as the degree of malignant transformation increases. Lack of internal complexity generally indicates benignity. These tumors have a varied range of prognosis; for example, a low-grade appendiceal mucinous neoplasm is indicative of a good prognosis, while a mucinous tumor of the rectum is known to manifest at an early age with aggressive behavior and poorer prognosis compared with its nonmucinous counterpart. Online supplemental material is available for this article. ©RSNA, 2022.
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Affiliation(s)
- Pei-Kang Wei
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Mamta Gupta
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Leo L Tsai
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Karen S Lee
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Adrian M Jaramillo
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Martin P Smith
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Jordan D LeGout
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
| | - Anuradha S Shenoy-Bhangle
- From the Departments of Radiology (P.K.W., L.L.T., K.S.L., M.P.S., A.S.S.B.) and Pathology (M.G.), Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215; Department of Radiology, Massachusetts General Hospital, Boston, Mass (A.M.J.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (J.D.L.)
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27
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Noguchi R, Yoshimatsu Y, Sin Y, Ono T, Tsuchiya R, Yoshida H, Kiyono T, Yonemura Y, Kondo T. Establishment and Characterization of NCC-PMP1-C1: A Novel Patient-Derived Cell Line of Metastatic Pseudomyxoma Peritonei. J Pers Med 2022; 12:258. [PMID: 35207746 PMCID: PMC8877412 DOI: 10.3390/jpm12020258] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2021] [Revised: 01/20/2022] [Accepted: 02/07/2022] [Indexed: 02/06/2023] Open
Abstract
Pseudomyxoma peritonei (PMP) is the intraperitoneal accumulation of mucus due to a mucinous tumor. PMP predominantly occurs in low-grade carcinomas. The incidence rate of PMP is one to two cases per million people per year. The standard therapy of PMP comprises complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. PMP recurs in about 50% of patients, and 30-40% are unable to receive the standard treatment because of its invasiveness. Therefore, novel therapies are of the utmost necessity. For basic and pre-clinical research, patient-derived cell lines are essential resources. However, only two PMP cell lines have been reported. Thus, we established a novel PMP cell line from resected metastatic PMP tissue. The cell line, named NCC-PMP1-C1, was maintained for more than 5 months and was passaged 25 times. NCC-PMP1-C1 cells demonstrated multiple amplifications and deletions, slow growth, tumorigenic ability, and dissemination of tumor cells in nude mice. We also used NCC-PMP1-C1 cells to screen drugs, which demonstrated a significant response to daunorubicin HCl, homoharringtonine, mitomycin C, and ponatinib. The NCC-PMP1-C1 cell line is the first PMP cell line derived from metastasized tissue and will be a potential resource for basic and pre-clinical research of metastasized PMP.
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Affiliation(s)
- Rei Noguchi
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
| | - Yuki Yoshimatsu
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
| | - Yooksil Sin
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
| | - Takuya Ono
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
| | - Ryuto Tsuchiya
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
- Department of Orthopedic Surgery, Graduate School of Medicine, Chiba University, Chiba 263-8522, Japan
| | - Hiroshi Yoshida
- Department of Diagnostic Pathology, National Cancer Center Hospital, Tokyo 104-0045, Japan;
| | - Tohru Kiyono
- Exploratory Oncology Research and Clinical Trial Center, National Cancer Center, Kashiwa 277-8577, Japan;
| | - Yutaka Yonemura
- NPO to Support Peritoneal Surface Malignancy Treatment, Japanese/Asian School of Peritoneal Surface Oncology, Kyoto 600-8189, Japan;
- Peritoneal Surface Malignancy Center, Department of Regional Cancer Therapy, Kishiwada Tokushukai Hospital, Kishiwada 596-8522, Japan
- Peritoneal Surface Malignancy Center, Department of Regional Cancer Therapy, Kusatsu General Hospital, Shiga 525-8585, Japan
| | - Tadashi Kondo
- Division of Rare Cancer Research, National Cancer Center Research Institute, Tokyo 104-0045, Japan; (R.N.); (Y.Y.); (Y.S.); (T.O.); (R.T.)
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28
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Flood MP, Kong JC, Pham T, Waters PS, Soucisse M, Ramsay R, Wong HL, Mitchell C, Michael M, McCormick JJ, Warrier S, Akhurst T, Heriot A. Diagnostic performance of positron emission tomography in the staging of Pseudomyxoma peritonei. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2022; 48:1606-1613. [DOI: 10.1016/j.ejso.2022.02.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2021] [Revised: 01/09/2022] [Accepted: 02/01/2022] [Indexed: 10/19/2022]
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29
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Liang L, Fang J, Han X, Zhai X, Song Y, Lu Y, Zhang Q, Ma R. Prognostic Value of CEA, CA19-9, CA125, CA724, and CA242 in Serum and Ascites in Pseudomyxoma Peritonei. Front Oncol 2021; 11:594763. [PMID: 34733775 PMCID: PMC8558433 DOI: 10.3389/fonc.2021.594763] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2020] [Accepted: 09/20/2021] [Indexed: 12/27/2022] Open
Abstract
Purpose To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), CA19-9, CA724, and CA242 in serum and ascites of pseudomyxoma peritonei (PMP) patients and evaluate the predictive value of these elevated biomarkers in pathological grade, completeness of cytoreduction (CC), and survival. Methods From May 2009 to October 2019, a total of 512 patients diagnosed with PMP through pathology in Aerospace Center Hospital were enrolled. The serum and ascites tumor biomarkers were obtained. The diagnostic values between serum and ascites biomarkers in pathology and CC were compared by the receiver operating characteristic (ROC) curves. The correlation between pathology, cytoreduction, and biomarkers was calculated by univariate and multivariate logistic regression. The associations between different numbers of elevated biomarkers and survival status were examined using univariate and multivariate backward Cox proportional hazard regression models. Results The results showed that the areas under the ROC curves (AUROC) in the diagnosis of CC were 0.798 (95% CI: 0.760-0.836) and 0.632 (95% CI: 0.588-0.676) in serum and ascites biomarkers, respectively. The elevated serum and ascites biomarkers were independent risk factors for both pathology and CC. The 1-year, 3-year, and 5-year survival rates were 89.07%, 73.22%, and 66.94%, respectively. Longer survival was observed in patients who had less than two elevated serum biomarkers compared with those with 2-3 and 4-5 elevated serum biomarkers (p < 0.001). Conclusion CEA, CA125, CA19-9, CA724, and CA242 in serum and ascites can be used to judge the severity and predict the resectability. Furthermore, different numbers of elevated biomarkers can help determine the prognosis of PMP.
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Affiliation(s)
- Lei Liang
- Department of Ultrasound, Aerospace Center Hospital, Beijing, China
| | - Jingyang Fang
- Department of Ultrasound, Aerospace Center Hospital, Beijing, China
| | - Xuedi Han
- Department of Ultrasound, Aerospace Center Hospital, Beijing, China
| | - Xichao Zhai
- Department of Myxoma, Aerospace Center Hospital, Beijing, China
| | - Yan Song
- Department of Clinical Laboratory, Aerospace Center Hospital, Beijing, China
| | - Yiyan Lu
- Department of Pathology, Aerospace Center Hospital, Beijing, China
| | - Qian Zhang
- Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Ruiqing Ma
- Department of Myxoma, Aerospace Center Hospital, Beijing, China
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30
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Nikiforchin A, Gushchin V, Sittig M, Baron E, Lopez-Ramirez F, Nieroda C, Sardi A. Outcomes Following Cytoreductive Surgery with Hyperthermic Intraperitoneal Chemotherapy with and without Diaphragmatic Resection in Patients with Peritoneal Metastases. Ann Surg Oncol 2021; 29:873-882. [PMID: 34546479 DOI: 10.1245/s10434-021-10669-9] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2021] [Accepted: 08/01/2021] [Indexed: 12/24/2022]
Abstract
BACKGROUND Diaphragmatic resection (DR) is often required during cytoreductive surgery/hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) to achieve complete cytoreduction (CC). While CC provides the best survival, requiring a DR may indicate unfavorable tumor biology. We assessed how DR during CRS/HIPEC affects outcomes. METHODS A retrospective cohort study was conducted using a prospective single-center database from October 1994-May 2020. Peritoneal surface malignancy patients who underwent CRS/HIPEC with CC-0/1/2 were assigned to DR and NoDR groups. Survival was measured using the Kaplan-Meier method. Subgroup analysis was performed for patients with peritoneal cancer index (PCI) ≥ 20 to eliminate confounding of more extensive disease in DR. RESULTS Of 824 CRS/HIPECs, 774 were included: 134 DR and 640 NoDR. PCI was significantly higher in DR: 29 versus 21, p < 0.001. CC-0/1 rate was 89% in DR and 95% in NoDR (p = 0.003). Neither 100-day morbidity nor mortality differed between the groups (p = 0.355 and p = 1.000). Median follow-up was 64 months. Median overall survival (OS) was significantly lower in DR (32 vs. 96 months, p < 0.001). Subgroup analysis by tumor type in patients with PCI ≥ 20 showed significantly shorter OS in DR than NoDR in appendiceal (40 vs. 196 months, p < 0.001) and colorectal (14 vs. 23 months, p = 0.003), but not in ovarian tumors (32 vs. 42 months, p = 0.893), whereas median PCI did not differ among subgroups. CONCLUSIONS DR during CRS/HIPEC does not increase morbidity and mortality. It is associated with worse survival in appendiceal and colorectal tumors, even after adjusting for tumor burden but does not appear to impact ovarian cancer survival.
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Affiliation(s)
- Andrei Nikiforchin
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Vadim Gushchin
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Michelle Sittig
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Ekaterina Baron
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Felipe Lopez-Ramirez
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Carol Nieroda
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA
| | - Armando Sardi
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, 227 Saint Paul Place, Baltimore, MD, 21202, USA.
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Han XD, Zhou N, Lu YY, Xu HB, Guo J, Liang L. Pseudomyxoma peritonei originating from intestinal duplication: A case report and review of the literature. World J Clin Cases 2021; 9:7459-7467. [PMID: 34616812 PMCID: PMC8464479 DOI: 10.12998/wjcc.v9.i25.7459] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2020] [Revised: 07/08/2021] [Accepted: 07/19/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pseudomyxoma peritonei (PMP) is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals. It is typically characterized by a type of gelatinous ascites named “jelly belly”. Most cases of PMP occur in association with ruptured primary mucinous tumors of the appendix (90%). Periodically, PMP can originate from mucinous carcinomas at other sites, including the colorectum, gallbladder, and pancreas. However, unusual origin can occur, as noted in this case report.
CASE SUMMARY A 52-year-old woman had an unusual derivation of PMP from intestinal duplication. The patient complained of abdominal distension and increasing abdominal girth. Abdominal contrast-enhanced computed tomography showed a mass in the greater omentum located on the left side of the abdomen, likely to be a cystic mass of peritoneal origin. A PMP diagnosis was presumed based on the specific signs of the mass with flocculent and stripe-like echoes in ultrasound images. Ultrasound-guided percutaneous aspiration suggested a high likelihood of PMP. Once the PMP diagnosis was recognized, identification of the origin of the primary tumor was indicated. Thus, an exploratory laparoscopy was performed. In the absence of a primary tumor of appendix origin, the diagnosis of a low-grade mucinous neoplasm of intestinal duplication origin was finally confirmed by histopathology.
CONCLUSION PMP is secondary to mucinous carcinomas of the appendix mostly. This case resulted from an unusual derivation from intestinal duplication.
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Affiliation(s)
- Xue-Di Han
- Department of Ultrasound, Aerospace Center Hospital, Beijing 100049, China
| | - Nan Zhou
- Department of Ultrasound, Aerospace Center Hospital, Beijing 100049, China
| | - Yi-Yan Lu
- Department of Pathology, Aerospace Center Hospital, Beijing 100049, China
| | - Hong-Bin Xu
- Department of Myxoma, Aerospace Center Hospital, Beijing 100049, China
| | - Jun Guo
- Department of Ultrasound, Aerospace Center Hospital, Beijing 100049, China
| | - Lei Liang
- Department of Ultrasound, Aerospace Center Hospital, Beijing 100049, China
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J Sharma V, Bhana J, Lolohea S, Meikle F. How to do hyperthermic intrathoracic chemotherapy for thoracic pseudomyxoma peritonei. ANZ J Surg 2021; 92:868-869. [PMID: 34396656 DOI: 10.1111/ans.17134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2021] [Revised: 07/21/2021] [Accepted: 08/01/2021] [Indexed: 11/30/2022]
Affiliation(s)
- Varun J Sharma
- Department of Cardiothoracic Surgery, Waikato District Health Board, Hamilton, New Zealand.,Waikato Institute of Surgical Education and Research, Hamilton, New Zealand
| | - Jack Bhana
- Department of Cardiothoracic Surgery, Waikato District Health Board, Hamilton, New Zealand
| | - Simione Lolohea
- Department of General Surgery, Waikato District Health Board, Hamilton, New Zealand
| | - Felicity Meikle
- Department of Cardiothoracic Surgery, Waikato District Health Board, Hamilton, New Zealand.,Waikato Institute of Surgical Education and Research, Hamilton, New Zealand
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Nikiforchin A, Gushchin V, King MC, Baron E, Lopez-Ramirez F, Sardi A. Cytoreductive Surgery with Hyperthermic Intrathoracic Chemotherapy for Patients with Intrapleural Dissemination of Peritoneal Surface Malignancies. Ann Surg Oncol 2021; 28:9126-9135. [PMID: 34263367 DOI: 10.1245/s10434-021-10298-2] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Accepted: 05/24/2021] [Indexed: 01/12/2023]
Abstract
BACKGROUND Peritoneal surface malignancies (PSM) can disseminate into the pleural cavity, increasing morbidity and mortality. While cytoreductive surgery with hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) improves outcomes for PSM with intra-abdominal spread, the optimal approach for patients with pleural dissemination from PSM remains unclear. It seems reasonable to apply peritoneal carcinomatosis management principles to patients with pleural lesions using CRS and hyperthermic intrathoracic chemotherapy (HITHOC). METHODS We conducted a descriptive study to evaluate outcomes of PSM patients who underwent CRS/HITHOC for pleural dissemination using a high-volume PSM center's prospective database from October 1994-June 2020. CRS/HITHOC was performed via either diaphragmatic window during CRS/HIPEC (CRS/HIPEC+HITHOC) or thoracotomy as a separate procedure (CRS/HITHOC). RESULTS Of 852 completed CRS/HIPECs, 18 HITHOCs in 15 patients were identified: 10 CRS/HIPEC+HITHOCs, and 8 CRS/HITHOCs. CRS/HIPEC+HITHOC primary tumors included: 4 appendix, 4 ovary, 1 colon, and 1 unknown. All (n = 8) CRS/HITHOC patients had recurrent appendiceal neoplasms. Complete cytoreduction was achieved in 90% of CRS/HIPEC+HITHOCs and 75% of CRS/HITHOCs. Major complications occurred in 20% of CRS/HIPEC+HITHOCs and 13% of CRS/HITHOCs with no 30-day mortality in either group. After median follow-up of 22 months, overall survival at 1, 3, and 5 years was 93.3%, 67.9%, and 67.9%, while 1-, 3-, and 5-year progression-free survival was 70.9%, 20.3%, and 20.3%. Intrapleural recurrence occurred in 1 CRS/HIPEC+HITHOC and 2 CRS/HITHOC patients. CONCLUSIONS CRS/HITHOC performed via diaphragm or thoracotomy at high-volume centers is a safe option for PSM with pleural dissemination. Further comparative studies with longer follow-up are needed to evaluate survival by tumor type.
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Affiliation(s)
- Andrei Nikiforchin
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Vadim Gushchin
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Mary Caitlin King
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Ekaterina Baron
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Felipe Lopez-Ramirez
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Armando Sardi
- Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA.
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Hishida T, Masai K, Kaseda K, Asakura K, Asamura H. Debulking surgery for malignant tumors: the current status, evidence and future perspectives. Jpn J Clin Oncol 2021; 51:1349-1362. [PMID: 34254145 DOI: 10.1093/jjco/hyab107] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2021] [Accepted: 06/22/2021] [Indexed: 11/14/2022] Open
Abstract
Debulking surgery, also called cytoreductive surgery, is a resection of the tumor as much as possible and an intended incomplete resection for unresectable malignant tumors. Since the most important principle in surgical oncology is complete R0 resection, debulking surgery goes against the basic principle and obscures the concept of operability. However, debulking surgery has been advocated for various types of advanced malignant tumors, including gynecological cancers, urological cancers, gastrointestinal cancers, breast cancers and other malignancies, with or without adjuvant therapy. Positive data from randomized trials have been shown in subsets of ovarian cancer, renal cell carcinoma, colorectal cancer and breast cancer. However, recent trials for renal cell carcinoma, colorectal cancer and breast cancer have tended to show controversial results, mainly according to the survival improvement of nonsurgical systemic therapy alone. On the other hand, debulking surgery still has a therapeutic role for slow-growing and borderline malignant tumors, such as pseudomyxoma peritonei and thymomas. The recent understanding of tumor heterogeneity and clonal evolution responsible for malignancy and drug resistance indicates that select patients may obtain prolonged survival by the synergistic effect of debulking surgery and novel systemic therapy. This review aimed to describe the current status and evidence of debulking surgery in a cross-organ manner and to discuss future perspectives in the current era with advances in systemic therapy.
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Affiliation(s)
| | | | | | | | - Hisao Asamura
- Division of Thoracic Surgery, Department of Surgery, Keio University School of Medicine, Tokyo, Japan
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35
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Kusamura S, Barretta F, Yonemura Y, Sugarbaker PH, Moran BJ, Levine EA, Goere D, Baratti D, Nizri E, Morris DL, Glehen O, Sardi A, Barrios P, Quénet F, Villeneuve L, Gómez-Portilla A, de Hingh I, Ceelen W, Pelz JOW, Piso P, González-Moreno S, Van Der Speeten K, Deraco M. The Role of Hyperthermic Intraperitoneal Chemotherapy in Pseudomyxoma Peritonei After Cytoreductive Surgery. JAMA Surg 2021; 156:e206363. [PMID: 33502455 DOI: 10.1001/jamasurg.2020.6363] [Citation(s) in RCA: 101] [Impact Index Per Article: 25.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Importance Studies on the prognostic role of hyperthermic intraperitoneal chemotherapy (HIPEC) in pseudomyxoma peritonei (PMP) are currently not available. Objectives To evaluate outcomes after cytoreductive surgery (CRS) and HIPEC compared with CRS alone in patients with PMP. Design, Setting, and Participants This cohort study analyzed data from the Peritoneal Surface Oncology Group International (PSOGI) registry, including 1924 patients with histologically confirmed PMP due to an appendiceal mucinous neoplasm. Eligible patients were treated with CRS with or without HIPEC from February 1, 1993, to December 31, 2017, and had complete information on the main prognostic factors and intraperitoneal treatments. Inverse probability treatment weights based on the propensity score for HIPEC treatment containing the main prognostic factors were applied to all models to balance comparisons between the CRS-HIPEC vs CRS-alone groups in the entire series and in the following subsets: optimal cytoreduction, suboptimal cytoreduction, high- and low-grade histologic findings, and different HIPEC drug regimens. Data were analyzed from March 1 to June 1, 2018. Interventions HIPEC including oxaliplatin plus combined fluorouracil-leucovorin, cisplatin plus mitomycin, mitomycin, and other oxaliplatin-based regimens. Main Outcomes and Measures Overall survival, severe morbidity (determined using the National Cancer Institute Common Terminology for Adverse Events, version 3.0), return to operating room, and 30- and 90-day mortality. Differences in overall survival were compared using weighted Kaplan-Meier curves, log-rank tests, and Cox proportional hazards multivariable models. A sensitivity analysis was based on the E-value from the results of the main Cox proportional hazards model. Differences in surgical outcomes were compared using weighted multivariable logistic models. Results Of the 1924 patients included in the analysis (997 [51.8%] men; median age, 56 [interquartile range extremes (IQRE), 45-65] years), 376 were in the CRS-alone group and 1548 in the CRS-HIPEC group. Patients with CRS alone were older (median age, 60 [IQRE, 48-70] vs 54 [IQRE, 44-63] years), had less lymph node involvement (14 [3.7%] vs 119 [7.7%]), received more preoperative systemic chemotherapy (198 [52.7%] vs 529 [34.2%]), and had higher proportions of high-grade disease (179 [47.6%] vs 492 [31.8%]) and suboptimal cytoreduction residual disease (grade 3, 175 [46.5%] vs 117 [7.6%]). HIPEC was not associated with a higher risk of worse surgical outcomes except with mitomycin, with higher odds of morbidity (1.99; 95% CI, 1.25-3.19; P = .004). HIPEC was associated with a significantly better overall survival in all subsets (adjusted hazard ratios [HRs], 0.60-0.68, with 95% CIs not crossing 1.00). The weighted 5-year overall survival was 57.8% (95% CI, 50.8%-65.7%) vs 46.2% (95% CI, 40.3%-52.8%) for CRS-HIPEC and CRS alone, respectively (weighted HR, 0.65; 95% CI, 0.50-0.83; P < .001; E-value, 2.03). Such prognostic advantage was associated with oxaliplatin plus fluorouracil-leucovorin (HR, 0.42; 95% CI, 0.19-0.93; P = .03) and cisplatin plus mitomycin (HR, 0.57; 95% CI, 0.42-0.78; P = .001) schedules. Conclusions and Relevance In this cohort study, HIPEC was associated with better overall survival when performed after CRS in PMP, generally without adverse effects on surgical outcomes.
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Affiliation(s)
- Shigeki Kusamura
- Peritoneal Surface Malignancies Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Istituto Nazionale Tumori dei Tumori di Milano, Milano, Italy
| | - Francesco Barretta
- Clinical Epidemiology and Trial Organization Unit, Fondazione IRCCS Istituto Nazionale Tumori dei Tumori di Milano, Milano, Italy
| | - Yutaka Yonemura
- Nonprofit Organization to Support Peritoneal Surface Malignancy Treatment, Kishiwada, Japan
| | | | - Brendan John Moran
- Basingstoke and North Hampshire National Health Service Foundation Trust, Basingstoke, United Kingdom
| | - Edward A Levine
- Surgical Oncology Service, Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina
| | - Diane Goere
- Department of Visceral and Oncologic Surgery, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France
| | - Dario Baratti
- Peritoneal Surface Malignancies Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Istituto Nazionale Tumori dei Tumori di Milano, Milano, Italy
| | - Eran Nizri
- Peritoneal Surface Malignancies Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Istituto Nazionale Tumori dei Tumori di Milano, Milano, Italy.,fellow at European School of Peritoneal Surface Oncology, Milano, Italy
| | - David Lawson Morris
- Hepatobiliary and Surgical Oncology Unit, Department of Surgery, University of New South Wales, St George Hospital, Sydney, Australia
| | - Olivier Glehen
- Department of Digestive Surgery, Centre Hospitalo-Universitaire Lyon-Sud, Hospices Civils de Lyon, Lyon, France
| | - Armando Sardi
- Division of Surgery, Department of Surgical Oncology, The Institute for Cancer Care, Mercy Medical Center, Baltimore, Maryland
| | - Pedro Barrios
- Department of Oncological Surgery, Hospital Sant Joan Despí, Moises Broggi, Peritoneal Surface Malignancy Catalonian's Programme, Sant Joan Despí, Barcelona, Spain
| | - François Quénet
- Centre Régional de Lutte Contre le Cancer Val d'Aurell, Montpellier, France
| | - Laurent Villeneuve
- Réseau National de Prise en Charge des Tumeurs Rares du Péritoine, French National Registry of Rare Peritoneal Surface Malignancies, Lyon, France
| | - Alberto Gómez-Portilla
- Department of General Surgery, Hospital Universitario de Araba, Hospital Universitario Araba Sede Hospital Santiago, Santiago, Spain.,Departamento de Cirugía General, Universidad del País Vasco, Vitoria, Spain.,Programa de Carcinomatosis Peritoneal, Hospital San José, Vitoria, Spain
| | - Ignace de Hingh
- Department of Surgery, Catharina Hospital, Eindhoven, the Netherlands
| | - Wim Ceelen
- Department of Gastrointestinal Surgery, Ghent University Hospital, Ghent, Belgium
| | - Joerg O W Pelz
- Department of Surgery I, University of Wuerzburg, Wuerzburg, Germany
| | - Pompiliu Piso
- Department of General and Visceral Surgery, Krankenhaus Barmherzige Brüder, Regensburg, Germany
| | - Santiago González-Moreno
- Peritoneal Surface Oncology Program, Department of Surgical Oncology, MD Anderson Cancer Center, Madrid, Spain
| | | | - Marcello Deraco
- Peritoneal Surface Malignancies Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Istituto Nazionale Tumori dei Tumori di Milano, Milano, Italy
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Lee JL, Kim M, Kim J, Kim CW, Ha YJ, Kim SY, Cho DH, Kim JC. Evaluation of the significance of pseudomyxoma peritonei patients based on the Peritoneal Surface Oncology Group International (PSOGI) classification. Asian J Surg 2021; 44:848-853. [PMID: 33622598 DOI: 10.1016/j.asjsur.2021.01.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2020] [Revised: 12/21/2020] [Accepted: 01/04/2021] [Indexed: 11/28/2022] Open
Abstract
BACKGROUND Pseudomyxoma peritonei (PMP) is a rare disease characterized by mucinous ascites and deposits on the peritoneal surfaces. The study aimed to assess PMP patients according to the Peritoneal Surface Oncology Group International (PSOGI) classification, as a part of standardization of this rare disease. METHODS This retrospective study analyzed PMP patients who underwent surgery between January 2007 and December 2017. All histologic slides were re-evaluated and the clinical data were collected. According to the PSOGI, PMP was retrospectively classified into three categories: low-grade (LG-PMP), high-grade (HG-PMP), and signet-ring cells (SRC-PMP). The extent of peritoneal involvement was quantified by the peritoneal cancer index (PCI). The completeness of cytoreduction (CCR) was dichotomized as complete or incomplete. RESULTS Fifty-seven patients were included in this study, consisted of 39 patients with LG-PMP (74.0%), 14 with HG-PMP (20.8%), and 4 with SRC-PMP (5.2%). There was no operative mortality and major complications occurred in 24 patients (31.2%). The 5-year overall survival was 56.2% ± 8.1% for LG-PMP, 37.5% ± 12.1% for HG-PMP, and 25.0% ± 21.7% for SRC-PMP. Concerning CCR, the 5-year overall (complete: 59.5% ± 8.4% vs. incomplete: 12.7% ± 8.1%, p = 0.001) and disease-free survival (complete: 38.6% ± 8.9% vs. incomplete: 7.7% ± 6.8%, p = 0.001) were significantly different. In a multivariable analysis, PSOGI classification and CCR independently correlated with survival (p = 0.011 and 0.018, respectively). CONCLUSIONS The PSOGI classification provides prognostic stratification, hopefully requiring further validation including every single case of PMP established as a standard criteria.
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Affiliation(s)
- Jong Lyul Lee
- Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea; Institute of Innovative Cancer Research, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Meejeong Kim
- Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Jihun Kim
- Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Chan Wook Kim
- Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea; Institute of Innovative Cancer Research, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Ye Jin Ha
- Institute of Innovative Cancer Research, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Seon-Young Kim
- Medical Genomics Research Center, Korea Research Institute of Bioscience & Biotechnology, Daejeon, South Korea
| | - Dong-Hyung Cho
- School of Life Science, Kyungpook National University, Daegu, South Korea
| | - Jin Cheon Kim
- Department of Surgery, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea; Institute of Innovative Cancer Research, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea.
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Swain D, Mason G, Yates A, Burke S, Cecil T, Mohamed F, Dayal S, Tzivanakis A, Moran B. Outcomes of home parenteral nutrition in 34 patients with intestinal failure from recurrent or progressive peritoneal malignancy of gastro-intestinal tract origin. Eur J Clin Nutr 2020; 75:856-858. [PMID: 33221816 DOI: 10.1038/s41430-020-00810-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2020] [Revised: 10/16/2020] [Accepted: 11/09/2020] [Indexed: 11/09/2022]
Abstract
OBJECTIVES To investigate the outcomes of 34 patients with intestinal failure secondary to advanced peritoneal malignancy on home parenteral nutrition (HPN). METHODS A retrospective analysis of all known patients receiving HPN at any time between January 2012 and the 31st March 2020 registered in a high volume peritoneal malignancy surgical centre database. RESULTS The median duration of HPN for all patients was 309.5 days (range 31-2198). Overall 11/34 went on to have multivisceral transplants. Of these 5/11 resumed normal oral intake off HPN, 3 died and 3 required ongoing HPN. Average time on HPN for patients with pseudomyxoma peritonei of appendix origin was 338 days (71-2198) compared with 90 days (31-260) in the group with more aggressive tumours. CONCLUSIONS HPN is feasible and effective in selected patients with pseudomyxoma peritonei as either a bridge to transplant or definitive treatment. As expected, patients with more aggressive tumours fare worse.
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Affiliation(s)
- David Swain
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK.
| | - Gemma Mason
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Allison Yates
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Sara Burke
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Tom Cecil
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Faheez Mohamed
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Sanjeev Dayal
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Alexios Tzivanakis
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
| | - Brendan Moran
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Aldermaston Rd, Basingstoke, RG24 9NA, UK
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Zhou S, Zhao H, He X. The Prognostic Impact of Pathology on Patients With Pseudomyxoma Peritonei Undergoing Debulking Surgery: A Systematic Review and Meta-Analysis of Retrospective Studies. Front Surg 2020; 7:554910. [PMID: 33304920 PMCID: PMC7701122 DOI: 10.3389/fsurg.2020.554910] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Accepted: 09/04/2020] [Indexed: 12/29/2022] Open
Abstract
Background: Pseudomyxoma peritonei (PMP) is a rare clinical condition with fatal outcomes, which is characterized by the progressive accumulation of mucinous ascites and peritoneal implants. Some studies have reported the effect of PMP biology on patient outcome. The objective of this study was to analyze published articles focusing on the impact of pathology on the prognosis of PMP patients undergoing debulking. Methods: Data from all studies regarding the prognosis of patients, with different pathologies, who underwent debulking surgery were analyzed. We searched PubMed, the Wiley Online Library, Ovid, and the Cochrane Library (through January 2020). Studies were confined to those articles written in English. Five studies were identified, and the differences in 5-year survival rates were analyzed according to the Kaplan–Meier survival curves. The hazard ratios (HRs) of the 5-year survival rates were calculated. Results: The mean and median 5-year survival rates of all patients were 39 and 40%, respectively. The median overall survival was 49.3 months. The mean 5-year survival rates of low-grade PMP was 45.2%. The five studies had sufficient data to calculate HRs from the 5-year survival rates data, and three had HRs lower than 1. The total HRs was 0.54, with a 95% CI between 0.33 and 0.89 (P = 0.01). Conclusions: Among PMP patients receiving debulking surgery who are not able to undergo complete cytoreductive surgery, low-grade biological PMP had a better prognosis than high-grade PMP.
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Affiliation(s)
- Shengnan Zhou
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
| | - Huaiyu Zhao
- Department of Surgery, Fuwai Hospital Shenzhen Center, China Academy of Medical Science, Shenzhen, China
| | - Xiaodong He
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China
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Mode of Presentation in 1070 Patients With Perforated Epithelial Appendiceal Tumors, Predominantly with Pseudomyxoma Peritonei. Dis Colon Rectum 2020; 63:1257-1264. [PMID: 33216496 DOI: 10.1097/dcr.0000000000001682] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
BACKGROUND Perforated epithelial appendiceal tumors are uncommon and can give rise to pseudomyxoma peritonei. Pseudomyxoma peritonei is rare, almost always of appendiceal origin, and presents in various ways relevant to abdominal surgeons. OBJECTIVE The aim of this study was to report the mode of presentation of pseudomyxoma peritonei of appendiceal origin in patients treated in a high-volume UK peritoneal malignancy center. DESIGN A retrospective observational study was conducted. SETTINGS This was a single-center study. PATIENTS Retrospective analysis of a prospective database of consecutive patients undergoing surgery between March 1994 and December 2016 was performed. MAIN OUTCOME MEASURES Mode of presentation was classified into 7 categories: "histological diagnosis at abdominal surgery for presumed appendicitis," "probable pseudomyxoma peritonei" based on abnormality on cross-sectional imaging (± image-guided biopsy)," "abnormal imaging (other cause suspected) and operative finding of pseudomyxoma peritonei," "diagnostic laparoscopy," "ovarian mass," "new-onset hernia," and "miscellaneous." RESULTS Overall, 1070 patients underwent surgery (female 61%, male 39%); median age was 57. The mode of presentation was abnormality on cross-sectional imaging in 324 of 1070 patients (30.3%), histological diagnosis at emergency surgery for presumed appendicitis in 203 of 1070 patients (19%), and abnormal imaging with eventual pseudomyxoma peritonei diagnosis in 180 of 1070 patients (16.8%); 124 of 651 women (19.2%) presented with an ovarian mass. New-onset hernia was the presenting feature in 9.9%; 83 of 1070 patients (7.7%) were diagnosed at diagnostic laparoscopy, and 32 of 1070 patients (3%) were described as "miscellaneous." Overall, 775 of 1070 patients (72.4%) had complete cytoreductive surgery with 10-year survival of 63.5%. LIMITATIONS Results are limited by the retrospective nature of the study, radiological improvements over the study period, and the number of patients who had overlapping features at presentation. CONCLUSION Perforated appendiceal tumors present in various ways but predominantly at cross-sectional imaging or coincidentally at laparoscopy or laparotomy. All abdominal surgeons will occasionally encounter cases, often unexpectedly, and recognition and referral to a specialized unit results in excellent outcomes in most cases. See Video Abstract at http://links.lww.com/DCR/B256. MODO DE PRESENTACIÓN EN 1070 PACIENTES CON TUMORES EPITELIALES APENDICULARES PERFORADOS, PREDOMINANTEMENTE CON PSEUDOMIXOMA PERITONEAL: Los tumores epiteliales apendiculares perforados son poco frecuentes y pueden dar lugar a Pseudomyxoma peritonei. El pseudomixoma peritoneal es raro, casi siempre de origen apendicular, y se presenta de diversas formas relevantes para los cirujanos abdominales.El objetivo fue informar el modo de presentación del Pseudomixoma peritoneal de origen apendicular en pacientes tratados en un centro de malignidad peritoneal de alto volumen en el Reino Unido.Se realizó un estudio observacional retrospectivo.Este fue un estudio de centro único.Análisis retrospectivo de una base de datos prospectiva de pacientes consecutivos sometidos a cirugía entre marzo de 1994 y diciembre de 2016.El modo de presentación se clasificó en 7 categorías: "diagnóstico histológico en la cirugía abdominal por presunta apendicitis", "probable Pseudomixoma peritoneal" basado en la anormalidad en la imagen de corte transversal (biopsia guiada por imagen +/-), "imagen anormal (se sospecha otra causa) y hallazgo quirúrgico de Pseudomixoma peritoneal", "laparoscopia diagnostica", "masa ovarica", "hernia de reciente aparicion" y "varios".En total, 1070 pacientes fueron operados (mujeres 61%, hombres 39%); edad media 57. El modo de presentación fue anormalidad en la imágen transversal en 324/1070 (30.3%), el diagnóstico histológico en cirugía de emergencia por presunta apendicitis en 203/1070 (19%), la imágen anormal con eventual diagnóstico de Pseudomixoma peritoneal en 180 / 1070 (16.8%), 124/651 (19.2%) las mujeres presentaron una masa ovárica. La hernia de reciente aparición fue la característica de presentación en 9.9%, 83/1070 (7.7%) fueron diagnosticados por laparoscopia diagnóstica y 32/1070 (3%) "misceláneos". En general, 775/1070 (72,4%) se sometió a cirugía citorreductora completa con una supervivencia a 10 años del 63,5%.Una deficiencia es que los resultados están limitados por la naturaleza retrospectiva del estudio, las mejoras radiológicas a lo largo del período de estudio, y varios pacientes tenían características superpuestas en la presentación.Los tumores apendiculares perforados se presentan de varias maneras, pero predominantemente en imágenes transversales o casualmente en laparoscopia o laparotomía. Todos los cirujanos abdominales encontrarán ocasionalmente con casos, a menudo inesperados, y el reconocimiento y la derivación a una unidad especializada da lugar a excelentes resultados en la mayoría de los casos. Consulte Video Resumen en http://links.lww.com/DCR/B256.).
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Kataoka A, Ito K, Takemura N, Inagaki F, Mihara F, Gohda Y, Kiyomatsu T, Yamada K, Kojima N, Igari T, Yamakawa M, Yano H, Kokudo N. Immunohistochemical staining as supportive diagnostic tool for pseudomyxoma peritonei arising from intraductal papillary mucinous neoplasm: A report of two cases and literature review. Pancreatology 2020; 20:1226-1233. [PMID: 32768178 DOI: 10.1016/j.pan.2020.06.008] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2020] [Revised: 06/09/2020] [Accepted: 06/10/2020] [Indexed: 12/11/2022]
Abstract
BACKGROUND/OBJECTIVES Pseudomyxoma peritonei (PMP) arising from an intraductal papillary mucinous neoplasm of the pancreas (IPMN) is a rare condition. The diagnosis of IPMN as the origin of PMP is mainly inferred from the clinical course and the exclusion of PMP from other organs. The pathological diagnosis has not yet been established. To evaluate the usefulness of immunohistochemical staining for the diagnosis of the primary lesion of PMP as IPMN. METHODS There are 2 cases of PMP arising from IPMN between March 2010 and December 2019 at National Center for Global Health and Medicine. A PubMed search that reported PMP arising from IPMN identified 16 additional cases. Diagnostic methods and clinicopathological features of 18 cases were compared. RESULTS Four cases including our two cases used immunohistochemical staining for the diagnosis of PMP arising from IPMN. The correspondence of the immunohistochemical staining between PMP and IPMN was shown in the three cases including previously reported two cases and one of our two cases to identify the primary lesion of PMP as IPMN. In addition, we revealed that the comparison of the immunostaining pattern of PMP with the representative immunostaining pattern of the candidate primary lesions is helpful for the diagnosis of the primary lesion of PMP. CONCLUSIONS Immunohistochemical staining is helpful to identify the primary lesion of PMP as IPMN.
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Affiliation(s)
- Atsuko Kataoka
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan; Course of Advanced and Specialized Medicine, Juntendo University Graduate School of Medicine, 2-1-1 Hongou, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Kyoji Ito
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Nobuyuki Takemura
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
| | - Fuyuki Inagaki
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Fuminori Mihara
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Yoshimasa Gohda
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Tomomichi Kiyomatsu
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Kazuhiko Yamada
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan; Course of Advanced and Specialized Medicine, Juntendo University Graduate School of Medicine, 2-1-1 Hongou, Bunkyo-ku, Tokyo, 113-8421, Japan
| | - Naoki Kojima
- Department of Pathology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Toru Igari
- Department of Pathology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Mitsunori Yamakawa
- Department of Pathology, Yamagata University, 2-2-2 Iidanishi, Yamagata-shi, Yamagata, 990-9585, Japan
| | - Hideaki Yano
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
| | - Norihiro Kokudo
- Department of Surgery, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan
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Chen P, Su L, Yang W, Zhang J, Wang Y, Wang C, Yu Y, Yang L, Zhou Z. Development and validation of prognostic nomograms for pseudomyxoma peritonei patients after surgery: A population-based study. Medicine (Baltimore) 2020; 99:e20963. [PMID: 32756083 PMCID: PMC7402788 DOI: 10.1097/md.0000000000020963] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND The aim of study was to develop and validate nomograms for predicting overall survival (OS) and cancer-specific survival (CSS) of patients with pseudomyxoma peritonei (PMP) and compare the predictive accuracy with the American Joint Committee on Cancer (AJCC) staging system. METHODS Data of 4959 PMP patients who underwent surgical resection were collected between 2004 and 2015 from the Surveillance Epidemiology and End Results (SEER) database. All included patients were divided into training (n = 3307) and validation (n = 1652) cohorts. The Kaplan-Meier method and Cox proportional hazard model were applied. Nomograms were validated by discrimination and calibration. Finally, concordance index (C-index) was used to compare the predictive performance of nomograms with that of the AJCC staging system. RESULTS According to the univariate and multivariate analyses of training sets, both nomograms for predicting OS and CSS combining age, grade, location, N stage, M stage, and chemotherapy were identified. Nomograms predicting OS also incorporated T stage and the number of lymph nodes removed (LNR). The calibration curves showed good consistency between predicted and actual observed survival. Moreover, C-index values demonstrated that the nomograms predicting both OS and CSS were superior to the AJCC staging system in both cohorts. CONCLUSION We successfully developed and validated prognostic nomograms for predicting OS and CSS in PMP patients. Two nomograms were more accurate and applicable than the AJCC staging system for predicting patient survival, which may help clinicians stratify patients into different risk groups, tailor individualized treatment, and accurately predict patient survival in PMP.
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Affiliation(s)
- Peng Chen
- Department of Gastrointestinal Surgery
| | | | | | | | - Yong Wang
- Department of Gastrointestinal Surgery
- Institute of Digestive Surgery, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital of Sichuan University, Chengdu
| | - Cun Wang
- Department of Gastrointestinal Surgery
- Institute of Digestive Surgery, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital of Sichuan University, Chengdu
| | - Yongyang Yu
- Department of Gastrointestinal Surgery
- Institute of Digestive Surgery, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital of Sichuan University, Chengdu
| | - Lie Yang
- Department of Gastrointestinal Surgery
- Institute of Digestive Surgery, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital of Sichuan University, Chengdu
- Department of General Surgery, West China-Ziyang Hospital of Sichuan University/The First People's Hospital of Ziyang, Ziyang, Sichuan Province, China
| | - Zongguang Zhou
- Department of Gastrointestinal Surgery
- Institute of Digestive Surgery, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital of Sichuan University, Chengdu
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Roy A, Stanford S, Nunn S, Alves S, Sargant N, Rangarajan S, Smith EA, Bell J, Dayal S, Cecil T, Tzivanakis A, Kruzhkova I, Solomon C, Knaub S, Moran B, Mohamed F. Efficacy of fibrinogen concentrate in major abdominal surgery - A prospective, randomized, controlled study in cytoreductive surgery for pseudomyxoma peritonei. J Thromb Haemost 2020; 18:352-363. [PMID: 31654548 PMCID: PMC7027898 DOI: 10.1111/jth.14665] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2019] [Revised: 10/18/2019] [Accepted: 10/22/2019] [Indexed: 01/12/2023]
Abstract
BACKGROUND Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei (PMP) is associated with excessive bleeding and acquired fibrinogen deficiency. Maintaining plasma fibrinogen may support hemostasis. OBJECTIVES To compare hemostatic efficacy and safety of human fibrinogen concentrate (HFC) vs cryoprecipitate as fibrinogen sources for bleeding patients with acquired fibrinogen deficiency undergoing PMP CRS. METHODS FORMA-05 was an off-label single-center, prospective, randomized, controlled phase 2 study. Patients undergoing PMP surgery with predicted intraoperative blood loss ≥2 L received human fibrinogen concentrate (HFC; 4 g) or cryoprecipitate (two pools of 5 units, containing approximately 4.0-4.6 g fibrinogen), repeated as needed. The primary endpoint was a composite of intraoperative and postoperative efficacy, graded using objective 4-point scales and adjudicated by an independent committee. RESULTS One hundred percent of patients receiving HFC (95% confidence interval: 83.9-100.0, n = 21) or cryoprecipitate (84.6-100.0, n = 22) achieved hemostatic success. HFC demonstrated noninferior efficacy (P = .0095; post hoc) and arrived in the operating room 46 minutes faster. There were significantly greater mean increases with HFC vs cryoprecipitate in plasma fibrinogen (0.78 vs 0.35 g/L; P < .0001) and FIBTEM A20 (3.33 vs 0.93 mm; P = .003). Factor XIII, factor VIII, and von Willebrand factor activity were maintained throughout surgery. Only red blood cells were transfused intraoperatively (median units: HFC group, 1.0; cryoprecipitate group, 0.5). Thromboembolic events were detected with cryoprecipitate only. Safety was otherwise comparable between groups. CONCLUSIONS Human fibrinogen concentrate was hemostatically efficacious in patients undergoing major abdominal PMP surgery, with a favorable safety profile. These results are relevant to other surgical settings where bleeding and acquired fibrinogen deficiency occur.
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Affiliation(s)
- Ashok Roy
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | | | - Sean Nunn
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | - Sue Alves
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | - Nigel Sargant
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | - Savita Rangarajan
- University Hospital Southampton NHS Foundation TrustUK
- KJ Somaiya AyurviharMumbaiIndia
| | | | - John Bell
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | - Sanjeev Dayal
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | - Tom Cecil
- Basingstoke and North Hampshire HospitalBasingstokeUK
| | | | | | | | | | - Brendan Moran
- Basingstoke and North Hampshire HospitalBasingstokeUK
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Yang SY, Kang JH, Kim HS, Han YD, Min BS, Lee KY. Status of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients with peritoneal carcinomatosis from colorectal cancer. J Gastrointest Oncol 2019; 10:1251-1265. [PMID: 31949946 PMCID: PMC6955007 DOI: 10.21037/jgo.2019.01.36] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2018] [Accepted: 01/27/2019] [Indexed: 12/19/2022] Open
Abstract
Peritoneal carcinomatosis (PC) was previously considered an incurable disease with a poor survival outcome. As our understanding of its biology evolved, the paradigm of the management of PC from colorectal cancer (CRC) has changed, including the combination of macroscopic disease control, cytoreductive surgery (CRS), maximal regional chemotherapy to treat residual microscopic disease, and hyperthermic intraperitoneal chemotherapy (HIPEC). As with many surgical innovations, CRS with HIPEC has evolved faster than data to support it, leaving many skeptics and critics. This review highlights the recent evidence of current practice and outcome of CRS with HIPEC. Furthermore, it also summarizes the ongoing clinical trials and potential future progress of this treatment modality.
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Affiliation(s)
- Seung Yoon Yang
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
| | - Jae Hyun Kang
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
| | - Ho Seung Kim
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
| | - Yoon Dae Han
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
| | - Byung Soh Min
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
| | - Kang Young Lee
- Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea
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Al-Azzawi M, Misdraji J, van Velthuysen MLF, Shia J, Taggart MW, Yantiss RK, Svrcek M, Carr N. Acellular mucin in pseudomyxoma peritonei of appendiceal origin: what is adequate sampling for histopathology? J Clin Pathol 2019; 73:220-222. [PMID: 31611287 DOI: 10.1136/jclinpath-2019-206213] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2019] [Accepted: 09/23/2019] [Indexed: 02/06/2023]
Abstract
INTRODUCTION Acellular intra-abdominal mucin is associated with a favourable prognosis in pseudomyxoma peritonei. There are no current guidelines on how many blocks are needed to classify the mucin as acellular with confidence. METHODS Specimens from cytoreductive surgery for mucinous appendiceal neoplasia, in which acellular mucin was found on initial histopathological examination, were prospectively identified. Additional tissue blocks were then taken to include either all residual visible intra-abdominal mucin or a maximum of 30 blocks. We also sent a questionnaire to pathologists in other centres. RESULTS Twelve patients were identified. In two cases, neoplastic epithelial cells were found on taking additional blocks. The questionnaire results suggested considerable variation in block-taking practice. CONCLUSION Taking additional tissue identified neoplastic cells in 2 of 12 cases. We recommend that sampling additional material should be considered when only acellular mucin is found on initial histology. Further work to determine the optimum sampling protocol is indicated.
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Affiliation(s)
- Marwa Al-Azzawi
- Department of Surgery, Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, UK
| | - Joseph Misdraji
- Depatment of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
| | | | - Jinru Shia
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - Melissa W Taggart
- Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Rhonda K Yantiss
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York city, New York, USA
| | - Magali Svrcek
- Department of Pathology, Hôpital Saint-Antoine, Paris, France
| | - Norman Carr
- Department of Pathology, Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, UK
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Sabesan A, Felder S, Feuerlein S, Lam C, McGettigan M, Powers BD, Dessureault S, Dineen SP. Preoperative Radiographic Assessment Predicts Incomplete Cytoreduction in Patients with Low Grade Mucinous Adenocarcinoma of the Appendix. Ann Surg Oncol 2019; 27:165-170. [DOI: 10.1245/s10434-019-07676-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2019] [Indexed: 12/29/2022]
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Cabanas J, Gomes da Silva R, Goldstein P, Verghese M, Sugarbaker PH. Recurrence of Pseudomyxoma Peritonei within the Inguinal Canal. TUMORI JOURNAL 2019; 91:481-6. [PMID: 16457146 DOI: 10.1177/030089160509100607] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Aim and Background Tumor appearing in an inguinal hernia sac indicates widespread carcinomatosis with ascites. A new onset hernia is a common clinical presentation of pseudomyxoma peritonei syndrome arising in an appendiceal mucinous tumor. Recurrence of pseudomyxoma peritonei within the inguinal region was previously reported in only a single patient. We present five patients with a recurrence of pseudomyxoma peritonei tumor nodules in the inguinal region following cytoreductive surgery and attempt to understand the etiology of this treatment failure. Methods and Study Design From a database of 910 patients with mucinous appendiceal tumors with peritoneal dissemination who had definitive treatment, five patients who had disease recurrence within the inguinal region were identified. The clinical histories were studied and presented in order to make future recommendations regarding the management of these patients. Results Five patients with ages ranging 39 to 67 years had a mucinous tumor in the inguinal region as a recurrence after a previous cytoreductive surgery combined with perioperative intraperitoneal chemotherapy. One of them had separate recurrence in both right and left inguinal regions. All these patients were made disease-free by the reoperative surgery with a sacrifice of the testicle in four. None have evidence of a recurrence of an inguinal hernia despite the lack of formal repair. Conclusion At the time of initial cytoreductive surgery the surgeon should be aware of the inguinal region as a possible site of relapse. This may be more problematic in patients who have had prior surgery with mucoid fluid in a hernia as the presenting sign of pseudomyxoma peritonei. Inguinal recurrences should be removed with clear margins even if orchiectomy is required.
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Cabanas J, Gomes da Silva R, Zappa L, Esquivel J, Cerruto C, Goldstein P, Sugarbaker PH. Splenic Metastases from Mucinous Neoplasms of the Appendix and Colon. TUMORI JOURNAL 2019; 92:104-12. [PMID: 16724688 DOI: 10.1177/030089160609200204] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Aims and Background Splenic metastases associated with mucinous intraabdominal tumors have been an enigma in the radiologic and oncology literature. These focal parenchymal defects from a non-metastasizing malignancy within an organ that rarely develops metastatic foci, even with high-grade cancer, were studied. Methods Information on 9 patients who underwent splenectomy with intraparenchymal splenic masses associated with appendiceal or colorectal mucinous tumors with peritoneal dissemination was collected. The CT scan, the histopathology and the clinical parameters of these patients were studied. A literature review searching for prior reports of this subject was performed. Results Eight of these patients had mucinous appendiceal tumors and 1 a mucinous sigmoid colon cancer. All patients had mucinous carcinomatosis at some time in their clinical course. These splenic tumor masses had a CT image compatible with metastases and not compatible with mucinous tumor layered out of the splenic capsule. None of the patients had evidence of metastases to other sites such as liver or lymph nodes. All patients had a mucinous histopathology. Splenectomy may be associated with prolonged survival. Conclusions From our review of the clinical information available on these 9 patients, these splenic lesions were thought to be an entrapment of mucinous tumor within splenic surface trabeculae, which expand into the splenic parenchyma resembling metastatic disease. These CT findings may be more accurately referred to as splenic pseudometastases.
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Chhabra P, Soni S, Khurana H. Pseudomyxoma Peritonei-An Unusual Cause of Ascites: A Case Report. Perm J 2019; 23:18-114. [PMID: 31314718 PMCID: PMC6636538 DOI: 10.7812/tpp/18-114] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
INTRODUCTION Ascites in patients with cardiac disease can be multifactorial. Serum ascitic albumin gradient (SAAG) helps in identifying the etiology of ascites. High SAAG ascites is related to hepatic or posthepatic causes. The causes of low SAAG ascites results include tuberculosis, peritoneal malignancy, or pancreatitis. CASE PRESENTATION We report an unusual cause of low SAAG ascites in a 48-year-old woman with valvular heart disease. The patient presented with ascites, and cross-sectional imaging revealed a right iliac fossa mass with omental deposits. The patient was finally diagnosed as having pseudomyxoma peritonei on the basis of clinicoradiopathological features. DISCUSSION Pseudomyxoma peritonei is a rare cause of low SAAG ascites. It is characterized by the deposition of mucinous material on the peritoneal surfaces. The most common site of origin is the appendix, although it can arise from other intraabdominal organs as well. Excision of the tumor combined with intraperitoneal chemotherapy is the preferred modality of treatment.
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Affiliation(s)
- Puneet Chhabra
- Department of Gastroenterology, Yashoda Superspeciality Hospital, Kaushambi, Ghaziabad, India
| | | | - Hunny Khurana
- Department of Medicine, Government Medical College and Hospital, Sector-32, Chandigarh, India
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Ning S, Yang Y, Wang C, Luo F. Pseudomyxoma peritonei induced by low-grade appendiceal mucinous neoplasm accompanied by rectal cancer: a case report and literature review. BMC Surg 2019; 19:42. [PMID: 31023277 PMCID: PMC6485155 DOI: 10.1186/s12893-019-0508-6] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2018] [Accepted: 04/09/2019] [Indexed: 11/25/2022] Open
Abstract
Background Pseudomyxoma peritonei (PMP) is a disease involving the peritoneum characterized by the production of large quantities of mucinous ascites. PMP has a low incidence, is difficult to diagnose, and has a guarded prognosis. PMP induced by low-grade appendiceal mucinous neoplasm is extremely rare, and PMP accompanied by rectal cancer is even rarer. Case presentation We present a unique case of a 70-year-old male with PMP induced by low-grade appendiceal mucinous neoplasm accompanied by rectal cancer. The patient’s clinical, surgical, and histologic data were reviewed. The patient had persistent distended abdominal pain without radiating lower back pain, abdominal distension for 1 month, and no exhaustion or defecation for 4 days. A transabdominal ultrasound-guided biopsy was performed on the first day. The patient received an emergency exploratory laparotomy because of increased abdominal pressure. We performed cytoreductive surgery, enterolysis, intestinal decompression, special tumour treatment and radical resection of rectal carcinoma. The postoperative course was uneventful. The postoperative histological diagnoses were PMP, low-grade appendiceal mucinous neoplasm and rectal medium differentiated adenocarcinoma. At the 1-year follow-up visit, no tumour recurrence was observed by computed tomography (CT). We also performed a literature review. Conclusions We should be aware that PMP can rarely be accompanied by rectal cancer, which represents an easily missed diagnosis and increases the difficulty of diagnosis and treatment. Additionally, there are some typical characteristics of PMP with respect to diagnosis and treatment.
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Affiliation(s)
- Shili Ning
- Department of General Surgery, The Second Hospital of Dalian Medical University, Zhongshan Road, Shahekou District, Dalian City, Liaoning Province, People's Republic of China, 116023
| | - Yanliang Yang
- Department of General Surgery, The Second Hospital of Dalian Medical University, Zhongshan Road, Shahekou District, Dalian City, Liaoning Province, People's Republic of China, 116023
| | - Chen Wang
- Department of General Surgery, The Second Hospital of Dalian Medical University, Zhongshan Road, Shahekou District, Dalian City, Liaoning Province, People's Republic of China, 116023
| | - Fuwen Luo
- Department of General Surgery, The Second Hospital of Dalian Medical University, Zhongshan Road, Shahekou District, Dalian City, Liaoning Province, People's Republic of China, 116023.
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Llueca A, Serra A, Maiocchi K, Delgado K, Jativa R, Gomez L, Escrig J. Predictive model for major complications after extensive abdominal surgery in primary advanced ovarian cancer. Int J Womens Health 2019; 11:161-167. [PMID: 30881145 PMCID: PMC6410758 DOI: 10.2147/ijwh.s190493] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Background Surgery for advanced ovarian cancer (AOC) frequently results in serious complications. The present study aimed to determine the importance of various factors and complications in cytoreductive surgery for AOC. Patients and methods The present study included 90 patients with AOC who underwent primary cytoreductive surgery in a single institution from January 2013 to August 2017. Demographic and clinicopathologic characteristics, surgical procedures, residual disease, and follow-up data were analyzed. Cytoreductive surgery was defined as complete (no residual tumor), optimal (residual tumor <1 cm in diameter), and suboptimal (residual tumor >1 cm in diameter). Grade III–IV complications were considered major. Patients were evaluated every 3–6 months. Results Surgical outcome was complete in 75 (82%), optimal in 5 (6%), and suboptimal in 11 (12%) patients. Major complications occurred in 28 (31%) patients. Independent risk factors for major complications were ≥five visceral resections, rectosigmoid resection, glissectomy, and pelvic peritonectomy. A score created by weighing the multivariate OR for each risk factor correctly predicted major complications in 67% of cases. A score cut-off of >2 discriminated between patients with and without complications in 79% of cases (95% CI: 70%–86%, P<0.001). Adjuvant chemotherapy was performed as planned in 67 patients (74%), including 50 (75%) without major complications and 17 (25%) with major complications. Conclusion Risk factors for major complications in cytoreductive surgery for AOC are ≥five visceral resections, rectosigmoid resection, glissectomy, and pelvic peritonectomy. Our model predicts morbidity based on major and minor classifications of complications.
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Affiliation(s)
- Antoni Llueca
- Department of Obstetrics and Gynaecology, University General Hospital of Castellon, Castellon, Spain, .,Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of Medicine, University Jaume I (UJI), Castellon, Spain,
| | - Anna Serra
- Department of Obstetrics and Gynaecology, University General Hospital of Castellon, Castellon, Spain, .,Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of Medicine, University Jaume I (UJI), Castellon, Spain,
| | - Karina Maiocchi
- Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of General Surgery, University General Hospital of Castellon, Castellon, Spain
| | - Katty Delgado
- Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of Radiology
| | - Rosa Jativa
- Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of Anaesthesiology, University General Hospital of Castellon, Castellon, Spain
| | - Luis Gomez
- Multidisciplinary Unit of Abdominal Pelvic Oncology Surgery (MUAPOS), University General Hospital of Castellon, Castellon, Spain, .,Department of General Surgery, University General Hospital of Castellon, Castellon, Spain
| | - Javier Escrig
- Department of Medicine, University Jaume I (UJI), Castellon, Spain, .,Department of General Surgery, University General Hospital of Castellon, Castellon, Spain.,Department of Radiology
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