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Chawrylak K, Leśniewska M, Mielniczek K, Sędłak K, Pelc Z, Kobiałka S, Pawlik TM, Polkowski WP, Rawicz-Pruszyński K. Current Status of Treatment among Patients with Appendiceal Tumors-Old Challenges and New Solutions? Cancers (Basel) 2024; 16:866. [PMID: 38473228 DOI: 10.3390/cancers16050866] [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: 01/21/2024] [Revised: 02/07/2024] [Accepted: 02/20/2024] [Indexed: 03/14/2024] Open
Abstract
The 5th edition of the World Health Organization (WHO) classification of tumors of the digestive system distinguishes four categories of appendiceal tumors (ATs): serrated lesions and polyps, mucinous neoplasms, adenocarcinomas, and neuroendocrine neoplasms (NENs). The differential diagnosis of ATs can be challenging in medical practice, due to their rarity and lack of data from randomized controlled trials on a large, diverse group of patients. ATs are usually noted in specimens obtained during appendectomies due to clinical acute appendicitis. In the European population, most ATs (65%) occur over the age of 50 and among women (56.8%). According to histological type, 54.6% are neuroendocrine tumors (NETs); 26.8% cystic, mucinous, and serous neoplasms; and 18.6% adenocarcinoma not otherwise specified (NOS). On pathologic analysis, most AT findings are benign lesions or small NENs that do not require further therapeutic measures. The presence of appendiceal mucinous neoplasm (AMN) can lead to pseudomyxoma peritonei (PMP). While the multimodal treatment for abdominal malignancies has evolved over the past several decades, the clinical workup and treatment of ATs remain a challenge. Therefore, this review aims to describe the diagnostic possibilities, molecular-based diagnosis, staging, differences in the treatment process, and prognostic factors associated with ATs.
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Affiliation(s)
- Katarzyna Chawrylak
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Magdalena Leśniewska
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Katarzyna Mielniczek
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Katarzyna Sędłak
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Zuzanna Pelc
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Sebastian Kobiałka
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Timothy M Pawlik
- Department of Surgery, The Ohio State University Wexner Medical Center and James Comprehensive Cancer Center, Columbus, OH 43210, USA
| | - Wojciech P Polkowski
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
| | - Karol Rawicz-Pruszyński
- Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland
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Sakhuja P, Aravindan KP. Neoplastic Lesions of Appendix. SURGICAL PATHOLOGY OF THE GASTROINTESTINAL SYSTEM 2022:895-921. [DOI: 10.1007/978-981-16-6395-6_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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3
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Abstract
Pathology is central to the management of peritoneal surface malignancy. This article highlights some recent advances that have had an impact on patient management or could do so in the near future. Malignant peritoneal mesothelioma, particularly the epithelioid subtype, is amenable to radical therapy in selected cases, and factors such as ki67 proliferation index, expression of BAP1 and mutation in CDKN2A show promise as prognostic indicators. Our understanding of multicystic mesothelioma has improved in recent years; it is a true neoplasm for which surgery may be indicated. Serous carcinomas involving the peritoneum are now known to originate from tubal epithelium. They are of two distinct types, high grade and low grade, which are now recognized as different neoplasms with distinctive features, oncogenesis and behavior. Pseudomyxoma peritonei (PMP) is an unusual condition that usually arises from an appendiceal mucinous neoplasm. Recent consensus in the classification and nomenclature of these lesions is discussed, including the distinction between low grade and high grade appendiceal mucinous neoplasms (HAMN), and the diagnostic criteria for appendiceal adenocarcinoma. PMP is divided into four prognostic groups: acellular mucin, low grade mucinous carcinoma peritonei, high grade mucinous carcinoma peritonei, and high grade mucinous carcinoma peritonei with signet ring cells. The pseudomyxoma microbiome is a promising area for clinical intervention but has been the subject of little research activity. Goblet cell adenocarcinoma (previously known as 'goblet cell carcinoid') is a distinctive type of appendiceal adenocarcinoma. Its behavior correlates with histologic features, but no general consensus for classification has been reached.
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Affiliation(s)
- Norman John Carr
- Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Basingstoke, UK
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4
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Delhorme JB, Severac F, Averous G, Glehen O, Passot G, Bakrin N, Marchal F, Pocard M, Lo Dico R, Eveno C, Carrere S, Sgarbura O, Quenet F, Ferron G, Goéré D, Brigand C. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei of appendicular and extra-appendicular origin. Br J Surg 2018; 105:668-676. [PMID: 29412465 DOI: 10.1002/bjs.10716] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/18/2017] [Revised: 07/29/2017] [Accepted: 09/03/2017] [Indexed: 12/11/2022]
Abstract
BACKGROUND The prognostic value of the primary neoplasm responsible for pseudomyxoma peritonei (PMP) remains poorly studied. The aim of this study was to determine the prognosis for patients with extra-appendicular PMP (EA-PMP) treated optimally with complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). METHODS All patients treated for PMP with CCRS and HIPEC between 1994 and 2016 were selected retrospectively from a French multicentre database. Patients with EA-PMP had pathologically confirmed non-neoplastic appendices and were matched in a 1 : 4 ratio with patients treated for appendicular PMP (A-PMP), based on a propensity score. RESULTS Some 726 patients were identified, of which 61 (EA-PMP group) were matched with 244 patients (A-PMP group). The origins of primary tumours in the EA-PMP group included the ovary (45 patients), colon (4), urachus (4), small bowel (1), pancreas (1) and unknown (6). The median peritoneal carcinomatosis index was comparable in EA-PMP and A-PMP groups (15·5 versus 18 respectively; P = 0·315). In-hospital mortality (3 versus 2·9 per cent; P = 1·000) and major morbidity 26 versus 25·0 per cent; P = 0·869) were also similar between the two groups. Median follow-up was 66·9 months. The 5-year overall survival rate was 87·8 (95 per cent c.i. 83·2 to 92·5) per cent in the A-PMP group and 87 (77 to 96) per cent in the EA-PMP group. The 5-year disease-free survival rate was 66·0 (58·7 to 73·4) per cent and 70 (53 to 83) per cent respectively. CONCLUSION Overall and disease-free survival following treatment with CCRS and HIPEC is similar in patients with pseudomyxoma peritonei of appendicular or extra-appendicular origin.
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Affiliation(s)
- J-B Delhorme
- Department of General and Digestive Surgery, Hautepierre Hospital, Strasbourg University Hospital, Strasbourg, France
| | - F Severac
- Department of Public Health, Strasbourg University Hospital, Strasbourg, France
| | - G Averous
- Department of Pathology, Hautepierre Hospital, Strasbourg University Hospital, Strasbourg, France
| | - O Glehen
- Department of General and Oncological Surgery, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France
| | - G Passot
- Department of General and Oncological Surgery, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France
| | - N Bakrin
- Department of General and Oncological Surgery, Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite, France
| | - F Marchal
- Department of Surgical Oncology, Alexis Vautrin Lorraine Institute of Oncology, Université de Lorraine, Vandoeuvre-lès-Nancy, France
| | - M Pocard
- Surgical Oncology and Digestive Unit, Lariboisière University Hospital, Paris, France
| | - R Lo Dico
- Surgical Oncology and Digestive Unit, Lariboisière University Hospital, Paris, France
| | - C Eveno
- Surgical Oncology and Digestive Unit, Lariboisière University Hospital, Paris, France
| | - S Carrere
- Department of Surgical Oncology, Montpellier Cancer Centre, Montpellier, France
| | - O Sgarbura
- Department of Surgical Oncology, Montpellier Cancer Centre, Montpellier, France
| | - F Quenet
- Department of Surgical Oncology, Montpellier Cancer Centre, Montpellier, France
| | - G Ferron
- Department of Surgical Oncology, Claudius Regaud Institute, Toulouse, France
| | - D Goéré
- Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif Cedex, France
| | - C Brigand
- Department of General and Digestive Surgery, Hautepierre Hospital, Strasbourg University Hospital, Strasbourg, France
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Abstract
Despite advances in our understanding of appendiceal mucinous neoplasms and their relationship to the pseudomyxoma peritonei syndrome, the classification of mucinous tumors of the appendix is still confusing. This review will provide an update on the various classification systems that have been recently proposed for appendiceal mucinous neoplasia, with a particular emphasis on how to handle and report the histologic findings for these tumors using the newly published Peritoneal Surface Oncology Group International (PSOGI) and American Joint Committee on Cancer (AJCC) eighth edition guidelines. A simplified approach to diagnostic reporting of appendiceal mucinous neoplasms based on the 3-tier AJCC grading scheme is detailed and specific criteria for assessing grade in appendiceal mucinous neoplasia will be outlined. In addition, histologic mimics of appendiceal mucinous neoplasia and how to distinguish these mimics from mucinous neoplasia will be discussed. Finally, despite improvements in diagnostic terminology, significant challenges in classifying appendiceal mucinous neoplasia persist and diagnostic strategies will be detailed to assist practicing pathologists in these challenging scenarios.
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Tan Y, Milikowski C, Toribio Y, Singer A, Rojas CP, Garcia-Buitrago MT. Intraductal papillary neoplasm of the bile ducts: A case report and literature review. World J Gastroenterol 2015; 21:12498-12504. [PMID: 26604656 PMCID: PMC4649132 DOI: 10.3748/wjg.v21.i43.12498] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2015] [Revised: 06/20/2015] [Accepted: 08/25/2015] [Indexed: 02/06/2023] Open
Abstract
Intraductal papillary neoplasm of the bile duct (IPNB) is a rare bile duct neoplasm mostly found in far eastern nations where hepatolithiasis and clonorchiasis infections are endemic. In western countries, it is very rare and the etiology is unknown. In this article, we report the first IPNB patient we encountered in our clinic and a literature review. The patient is a 38-year-old female with a history of choledocholithiasis who presented with obstructive jaundice. She was found to have a papillary mass at the junction of the right hepatic duct and common hepatic duct with six masses in the liver parenchyma. The immunophenotypic and histologic features of the tumor are consistent with IPNB, gastric subtype. The patient had a partial hepatectomy and has been receiving palliative chemotherapy. In a search of PubMed database, we collected 354 IPNB patients reported in 22 articles. In these patients, 52.8% were from Japan and 27.7% were from western countries including the United States (11.0%). The age of the patients ranged from 35 to 80 years old with an average of 64.6. Male/female ratio was 1.5. Macroscopically, 57.5% of the tumors were in the left lobe and 29.5% were in the right lobe. The average size of the tumor were 4.2 cm at the time of diagnosis. Histologically, pancreato-biliary subtype accounted for 41.8%, intestinal 28.0%, gastric 13.5% and oncocytic 16%. An invasive component is most often present in the pancreato-biliary and gastric subtypes. Despite recent advanced technologies, diagnosis of IPNB is still challenging, especially in western countries due to its rarity. Defined clinico-pathologic features are in demand for the accurate diagnosis and proper treatment.
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MESH Headings
- Adenocarcinoma, Papillary/chemistry
- Adenocarcinoma, Papillary/pathology
- Adenocarcinoma, Papillary/therapy
- Adult
- Bile Duct Neoplasms/chemistry
- Bile Duct Neoplasms/pathology
- Bile Duct Neoplasms/therapy
- Biomarkers, Tumor/analysis
- Biopsy
- Chemotherapy, Adjuvant
- Female
- Hepatectomy
- Hepatic Duct, Common/chemistry
- Hepatic Duct, Common/pathology
- Hepatic Duct, Common/surgery
- Humans
- Immunohistochemistry
- Magnetic Resonance Imaging
- Neoplasm Invasiveness
- Neoplasms, Multiple Primary/chemistry
- Neoplasms, Multiple Primary/pathology
- Neoplasms, Multiple Primary/therapy
- Palliative Care
- Tomography, X-Ray Computed
- Treatment Outcome
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Ruptured intrahepatic biliary intraductal papillary mucinous neoplasm in a Jehovah's Witness patient. Int Surg 2014; 99:590-4. [PMID: 25216426 DOI: 10.9738/intsurg-d-13-00134.1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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Kishida N, Shinoda M, Masugi Y, Itano O, Fujii-Nishimura Y, Ueno A, Kitago M, Hibi T, Abe Y, Yagi H, Tanimoto A, Tanabe M, Sakamaoto M, Kitagawa Y. Cystic tumor of the liver without ovarian-like stroma or bile duct communication: two case reports and a review of the literature. World J Surg Oncol 2014; 12:229. [PMID: 25047921 PMCID: PMC4119182 DOI: 10.1186/1477-7819-12-229] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2013] [Accepted: 07/04/2014] [Indexed: 02/07/2023] Open
Abstract
We report two cases of cystic neoplasm of the liver with mucinous epithelium in which both ovarian-like stroma and bile duct communication were absent. The first case was a 41-year-old woman. She underwent right trisegmentectomy due to a multilocular cystic lesion, 15 cm in diameter, with papillary nodular components in the medial segment and right lobe. Histologically, arborizing papillae were seen in the papillary lesion. The constituent neoplastic cells had sufficient cytoarchitectural atypia to be classified as high-grade dysplasia. The second case was a 60-year-old woman. She underwent left lobectomy due to a unilocular cystic lesion, 17 cm in diameter, in the left lobe. Histologically, the cyst wall was lined by low columnar epithelia with slight cellular atypia. In both cases, neither ovarian-like stroma nor bile duct communications were found throughout the resected specimen. According to the most recent World Health Organization (WHO) classification in 2010, cystic tumors of the liver with mucinous epithelium are classified as mucinous cystic neoplasms when ovarian-like stromata are found, and as intraductal papillary neoplasm of bile duct when bile duct communication exists. Therefore, we diagnosed the cystic tumors as ‘biliary cystadenoma’ according to the past WHO classification scheme from 2000. We believe that the combined absence of both ovarian-like stroma and bile duct communication is possible in mucinous cystic tumors of the liver. Herein, we have described the clinicopathologic features of the two cases and reviewed past cases in the literature.
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Affiliation(s)
| | - Masahiro Shinoda
- Department of Surgery, Keio University, School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
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Peng P, Keng S, Ming W, Huifang H, Linya P, Jiaxin Y. Female patients with pseudomyxoma peritonei: a single-institution clinicopathologic study of 35 cases. Arch Gynecol Obstet 2013; 289:365-72. [PMID: 23949422 DOI: 10.1007/s00404-013-3001-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2012] [Accepted: 08/05/2013] [Indexed: 11/28/2022]
Abstract
PURPOSE To explore clinicopathologic/prognostic aspects of pseudomyxoma peritonei (PMP). METHODS We reviewed records of 35 female patients with PMP treated at a single institution. RESULTS Patients' median age was 57.0 years (range 35.0-71.0 years). Their median pre-surgery level of carbohydrate antigen 19-9 (CA-199) was 80.95 U/ml (range 0.00-1,562.10 U/ml); of carbohydrate antigen 12-5 (CA-125), 44.00 U/ml (range 0.90-231.20 U/ml); and of carcinoembryonic antigen (CEA), 17.20 ng/ml (range 2.04-211.60 ng/ml). Of the 35 patients, 23 (65.7 %) underwent cytoreductive surgery (CRS) by gynecological oncologists and 12 (34.3 %) underwent non-CRS surgeries by general gynecologists or surgeons, including one patient who refused surgical treatment beyond a diagnostic laparoscopy. After surgery, 18 patients (51.4 %) had residual lesions, 11 (31.4 %) had complete lesion removal and 6 (17.1 %)had insufficient information on residual lesion; 21 (60.0 %) had appendiceal-based tumors and 12 (34.3 %) had ovarian-based tumors. Median follow-up time was 37 months (range 1-148 months), during which 28 patients (80.0 %) had relapsed. By the end of the study, 12 patients (34.3 %) died of PMP, 16 (45.7 %) survived with disease, and 7 (20 %) survived without disease. Median progress-free survival (PFS) was 12 months (range 0.5-114.0 months). Median overall survival time was 42 months (range 5-150 months). Ovarian tumor origin, post-surgery residual lesions, preoperative CA199 > 258.9 U/ml and CA125 > 70.6 U/ml were independent predictors of PFS. CONCLUSIONS PMP is rare in women, and has a poor long-term survival rate. Multi-center cooperation to gather more cases is needed to explore its behavior and proper treatment.
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Affiliation(s)
- Peng Peng
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Beijing, 100730, People's Republic of China,
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Castellano-Megías VM, Ibarrola-de Andrés C, Colina-Ruizdelgado F. Pathological aspects of so called "hilar cholangiocarcinoma". World J Gastrointest Oncol 2013; 5:159-170. [PMID: 23919110 PMCID: PMC3731529 DOI: 10.4251/wjgo.v5.i7.159] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2012] [Revised: 04/14/2013] [Accepted: 05/19/2013] [Indexed: 02/05/2023] Open
Abstract
Cholangiocarcinoma (CC) arising from the large intrahepatic bile ducts and extrahepatic hilar bile ducts share clinicopathological features and have been called hilar and perihilar CC as a group. However, “hilar and perihilar CC” are also used to refer exclusively to the intrahepatic hilar type CC or, more commonly, the extrahepatic hilar CC. Grossly, a major distinction can be made between papillary and non-papillary tumors. Histologically, most hilar CCs are well to moderately differentiated conventional type (biliary) carcinomas. Immunohistochemically, CK7, CK20, CEA and MUC1 are normally expressed, being MUC2 positive in less than 50% of cases. Two main premalignant lesions are known: biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the biliary tract (IPNB). IPNB includes the lesions previously named biliary papillomatosis and papillary carcinoma. A series of 29 resected hilar CC from our archives is reviewed. Most (82.8%) were conventional type adenocarcinomas, mostly well to moderately differentiated, although with a broad morphological spectrum; three cases exhibited a poorly differentiated cell component resembling signet ring cells. IPNB was observed in 5 (17.2%), four of them with an associated invasive carcinoma. A clear cell type carcinoma, an adenosquamous carcinoma and two gastric foveolar type carcinomas were observed.
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