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Liu Y, Peng J, Zhao Y, Wang W. Emerging pathological diagnostic strategies for solid pseudopapillary neoplasm of the pancreas: insights from omics and innovative techniques. J Pathol Clin Res 2025; 11:e70029. [PMID: 40312910 PMCID: PMC12046068 DOI: 10.1002/2056-4538.70029] [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: 12/11/2024] [Revised: 02/21/2025] [Accepted: 03/31/2025] [Indexed: 05/03/2025]
Abstract
Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant tumor, representing 0.9-2.7% of all exocrine pancreatic tumors. SPN patients generally have a favorable prognosis with a 5-year survival rate exceeding 95% following complete surgical resection. Accurate diagnosis is crucial to avoid unnecessary treatments. Currently, SPN diagnosis relies on imaging techniques such as CT and MRI, along with immunohistochemical analysis of biopsy and resection samples. The main challenge in diagnosis is the potential inability to accurately identify recurrent or metastatic SPN, as well as 'malignant' SPN, due to the lack of specific biomarkers. Advances in high-throughput omics technologies, including genomics, transcriptomics, proteomics and metabolomics, have opened new avenues for identifying novel biomarkers for SPN. Additional, liquid biopsy techniques have enabled more comprehensive analysis of biosamples such as pancreatic cyst fluid, offering promising prospects for preoperative diagnosis. This review highlights recent research on SPN diagnosis, focusing on immunohistochemical markers, tissue sampling methods and the potential of omics approaches. It also discusses the challenges and opportunities in improving diagnostic accuracy, particularly for high-grade and metastatic SPNs.
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Affiliation(s)
- Yuanhao Liu
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
| | - Junya Peng
- Institute of Clinical MedicinePeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
- State Key Laboratory of Complex, Severe, and Rare DiseasesPeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
| | - Yupei Zhao
- State Key Laboratory of Complex, Severe, and Rare DiseasesPeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
- Department of General SurgeryPeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
- Department of Basic Medical SciencesSchool of Medicine, Tsinghua UniversityBeijingPR China
- Peking University‐Tsinghua Center for Life SciencesBeijingPR China
| | - Wenze Wang
- Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical CollegeBeijingPR China
- Molecular Pathology Research Center, Department of PathologyPeking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingPR China
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Zhao S, Wang J, Liu H, Liu S, Sun L, Wang Y, Gao S, Sun Y. Analyses of CTNNB1 mutation and expression and clinicopathological characteristics in 179 cases of solid-pseudopapillary neoplasm of the pancreas. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2025; 32:327-338. [PMID: 39991945 DOI: 10.1002/jhbp.12123] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/25/2025]
Abstract
BACKGROUND Nuclear expression of CTNNB1 is occasionally negative in solid-pseudopapillary neoplasm (SPN) of the pancreas, leading to a missed diagnosis. In the present study, we aimed to investigate the clinical significance of CTNNB1 mutation detection for diagnosing SPN and explore the difference in clinicopathological characteristics at different ages and sex. METHODS Patients who underwent surgery for a pathologically confirmed SPN in our institution between 2011 and 2020 were collected. Their clinicopathological data were analyzed. RESULTS The median age of the 179 patients was 31 years (6-64 years), including 34 pediatric patients (19.0%), and 32 patients were male (17.9%). We detected point mutations in exon 3 of CTNNB1 in 74.3% (133/179) of SPNs by Sanger sequencing. The main mutation sites were D32, S33, S37, G34 and T41. In the three SPNs without nuclear expression of CTNNB1, Sanger sequencing showed point mutations of CTNNB1. NGS did not detect any consistent mutation except CTNNB1 in the three cases. The tumor size, Ki-67 index, and the negative rates of CTNNB1 nuclear expression and synaptophysin expression in the pediatric group were higher than those in other groups (p < .05). CONCLUSIONS For atypical cases, testing for CTNNB1 mutations can help in the accurate diagnosis of SPN. Compared with adult patients, pediatrics with SPN may be more prone to recurrence, and their immunohistochemical phenotype is more complex, requiring additional care in the diagnosis and postoperative follow-up.
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Affiliation(s)
- Shuai Zhao
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
| | - Jian Wang
- Department of Pancreatic Carcinoma, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Tianjin Key Laboratory of Digestive Cancer, Tianjin, China
| | - Huimin Liu
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
| | - Shasha Liu
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
| | - Lin Sun
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
| | - Ying Wang
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
| | - Song Gao
- Department of Pancreatic Carcinoma, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Tianjin Key Laboratory of Digestive Cancer, Tianjin, China
| | - Yan Sun
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, Tianjin's Clinical Research Center for Cancer, Tianjin, China
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Sapkota A, Paudel R, Pandey S, Bhatt N. Solid pseudopapillary neoplasm of the pancreas in an adolescent: A case report and review of the literature. World J Gastrointest Oncol 2025; 17:101859. [PMID: 40092930 PMCID: PMC11866218 DOI: 10.4251/wjgo.v17.i3.101859] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2024] [Revised: 11/27/2024] [Accepted: 12/19/2024] [Indexed: 02/14/2025] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare epithelial tumor that primarily affects young women. Since the condition is often asymptomatic or presents with non-specific symptoms, its diagnosis can be difficult. CASE SUMMARY This report details the case of a 15-year-old girl who presented with a 2-year history of abdominal pain, with no significant findings during physical examination. Abdominal ultrasound revealed a well-defined heterogeneous solid-cystic mass in the epigastric region, likely originating from the tail of the pancreas. A subsequent contrast-enhanced computed tomography scan indicated a well-defined cystic lesion with an enhancing solid component and capsule in the tail of the pancreas, suggestive of a cystic neoplasm. The patient underwent an open distal pancreatectomy with splenectomy, and histopathological analysis confirmed the diagnosis of SPN of the pancreas. CONCLUSION This case highlights the risk of SPN in adolescent girls and the necessity of early diagnosis and intervention for better outcomes.
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Affiliation(s)
- Aakriti Sapkota
- Department of Medicine, B.P. Koirala Institute of Health Sciences, Dharan 56700, Koshi, Nepal
| | - Rajesh Paudel
- Department of Radiodiagnosis and Imaging, B.P. Koirala Institute of Health Sciences, Dharan 56700, Koshi, Nepal
| | - Sandip Pandey
- Department of Medicine, B.P. Koirala Institute of Health Sciences, Dharan 56700, Koshi, Nepal
| | - Navin Bhatt
- Department of Medicine, NYC Health + Hospitals/Elmhurst, Icahn School of Medicine at Mount Sinai, Elmhurst, NY 11373, United States
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Zhao Z, Lin J, Bai T, Liao H, Liu Z. Case Report: Primary solid pseudopapillary neoplasm of the ovary with "cholesteroma-like" denaturation. Front Oncol 2025; 15:1514460. [PMID: 40115014 PMCID: PMC11922707 DOI: 10.3389/fonc.2025.1514460] [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: 10/20/2024] [Accepted: 02/10/2025] [Indexed: 03/22/2025] Open
Abstract
Solid pseudopapillary neoplasms (SPNs) primarily arise in the pancreas and are uncommon in the ovaries. Here, we present a case of ovarian-origin SPN. Alongside the typical solid and pseudopapillary structures, "cholesteroma-like" denaturation areas and tissue degeneration regions are also observed. Immunohistochemistry analysis demonstrates positive results for β-catenin (nucleus), CD99 (dot-like), CD56, and vimentin. Imaging studies rule out pancreatic or other origins. This study aims to enhance comprehension, diagnosis, and differential diagnosis of primary ovarian SPN among pathologists and clinicians, as well as to investigate the origin and management of primary solid pseudopapillary tumors in the ovary.
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Affiliation(s)
- Ziqing Zhao
- Department of Pathology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Jiahui Lin
- Department of Radiology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Tingting Bai
- Department of Pathology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Hongfeng Liao
- Department of Pathology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Zhengjin Liu
- Department of Pathology, Zhongshan Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
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Luchini C. Diagnostic Pearls and Pitfalls in the Evaluation of Biopsies of the Pancreas. Arch Pathol Lab Med 2025; 149:e54-e62. [PMID: 38387616 DOI: 10.5858/arpa.2023-0426-ra] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/13/2023] [Indexed: 02/24/2024]
Abstract
CONTEXT.— The examination of small pancreatic biopsies is a difficult task for pathologists. This is due to the scant and fragmented material often obtained from diagnostic procedures as well as the significant overlap between different neoplastic and nonneoplastic entities. In the upcoming neoadjuvant era, biopsies could become even more important, representing the only possibility to look at the real histomorphology of tumors before chemotherapy-induced modifications. OBJECTIVES.— To summarize and discuss the state-of-the-art diagnostic workflow for small pancreatic biopsies, including the most important morphologic and immunohistochemical features and molecular alterations. The main diagnostic pearls and pitfalls of this challenging scenario are also discussed. The most important topics of this review are represented by: (1) pancreatic ductal adenocarcinoma, along with its main differential diagnoses, including autoimmune pancreatitis; (2) solid hypercellular neoplasms, including neuroendocrine neoplasms, acinar cell carcinoma, pancreatoblastoma, and solid pseudopapillary neoplasms; and (3) cystic lesions. Real-world considerations will also be presented and discussed. DATA SOURCES.— Sources included a literature review of published studies and the author's own work. CONCLUSIONS.— The correct diagnosis of pancreatic lesions is a crucial step in the therapeutic journey of patients. It should be based on robust, standardized, and reliable hallmarks. As presented and discussed here, the integration of morphology with immunohistochemistry, and, in selected cases, with molecular analysis, represents a decisive step in this complex scenario.
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Affiliation(s)
- Claudio Luchini
- From the Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy; and the ARC-Net Research Center, University of Verona, Verona, Italy
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Maity R, Dhali A, Chattopadhyay PK, Biswas J, Dhali GK. Solid pseudopapillary neoplasm of the pancreas causing obstructive jaundice: Case report of a rare entity. Int J Surg Case Rep 2025; 126:110635. [PMID: 39612899 PMCID: PMC11636209 DOI: 10.1016/j.ijscr.2024.110635] [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: 10/06/2024] [Revised: 11/16/2024] [Accepted: 11/20/2024] [Indexed: 12/01/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasm (SPN) is an extremely rare, low-grade, malignant pancreatic tumour with an excellent prognosis. We describe a case of SPN causing obstructive jaundice in a young female, thus mimicking pancreatic adenocarcinoma clinically and radiologically. CASE PRESENTATION A 32-year-old female presented with abdominal pain for 12 h, icterus, and an epigastric mass measuring 3 × 3 cm. Imaging studies revealed features of pancreatic neoplasm in the head of pancreas, possibly pancreatic adenocarcinoma. However, ultrasound-guided fine-needle aspiration cytology of the lesion revealed were indicative of SPN. The patient was managed by biliary decompression and underwent Whipple procedure for complete tumour resection. Histopathological analysis of the resected specimen confirmed the diagnosis of SPN. DISCUSSION SPN commonly presents in young females with abdominal pain, nausea, and vomiting. Our patient presented with jaundice, which is a rare finding even for pancreatic head tumours. Her clinical and radiological findings indicated pancreatic adenocarcinoma, since it commonly presents with painless jaundice, abdominal pain, and abdominal mass. Diagnostic confirmation is achieved by tissue biopsy - the presence of pseudopapillary patterns is a characteristic feature of SPN. Contrary to pancreatic adenocarcinoma, SPN has an excellent prognosis. Complete tumour resection is the mainstay of treatment for SPN, even in cases of local invasion or metastasis. CONCLUSION SPN poses a diagnostic challenge due to its non-specific presentation. Clinicians should consider SPN as a differential in women presenting with abdominal mass and vague abdominal complaints. Complete surgical resection is the mainstay of treatment for SPN.
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Affiliation(s)
- Rick Maity
- Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
| | - Arkadeep Dhali
- Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India; Sheffield Teaching Hospitals NHS Foundation Trust, UK; School of Medicine and Population Health, University of Sheffield; School of Medicine, University of Leeds, UK; Deanery of Clinical Sciences, The University of Edinburgh, UK.
| | | | - Jyotirmoy Biswas
- College of Medicine and Sagore Dutta Hospital, Kolkata, West Bengal, India
| | - Gopal Krishna Dhali
- Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
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Xia R, Tang LH, Shafizadeh N, Xue Y, Liu X. Unveiling lipid-rich ("clear cell") pancreatic neuroendocrine tumor: A small series of two cases with literature review. Diagn Cytopathol 2024; 52:582-588. [PMID: 38426360 DOI: 10.1002/dc.25296] [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: 01/13/2024] [Revised: 02/17/2024] [Accepted: 02/19/2024] [Indexed: 03/02/2024]
Abstract
This study presents two cases of lipid-rich pancreatic neuroendocrine tumors (PanNETs), a rare variant posing significant diagnostic challenges in fine needle aspiration (FNA) cytology and small biopsies. The first case involves an elderly male with a pancreatic tumor, displaying distinct cytoplasmic vacuoles, while the second case is a middle-aged male present with a pancreatic tail mass exhibiting foamy cytoplasm and eccentric nuclei, infiltrating in the stroma. Both cases did not exhibit typical morphologic features of PanNET but demonstrated cytomorphologic features and infiltrative growth patterns that mimicked adenocarcinoma. Further work-up demonstrated that both tumors were immunoreactive for synaptophysin and chromogranin, and were interpreted as well-differentiated, PanNET, lipid-rich variant. The study highlights the overlapping morphological features between lipid-rich PanNETs and other pancreatic neoplasms and underscores the importance of comprehensive cytological and immunohistochemical analysis for accurately diagnosing this variant, particularly due to the risk of misinterpreting it as pancreatic adenocarcinoma. Recognizing lipid-rich PanNETs is crucial for appropriate clinical management, as their identification can significantly impact treatment decisions and patient outcomes.
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Affiliation(s)
- Rong Xia
- Department of Pathology, NYU Langone Health, New York, New York, USA
| | - Laura H Tang
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - Negin Shafizadeh
- Department of Pathology, NYU Langone Health, New York, New York, USA
| | - Yue Xue
- Department of Pathology, Case Western Reserve University, Cleveland, Ohio, USA
| | - Xiaoying Liu
- Department of Pathology and Lab Medicine, Dartmouth Health and Geisel School of Medicine at Dartmouth, Hanover, New Hampshire, USA
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Mazzarella G, Muttillo EM, Coletta D, Picardi B, Rossi S, Rossi Del Monte S, Gomes V, Muttillo IA. Solid pseudopapillary tumor of the pancreas: A systematic review of clinical, surgical and oncological characteristics of 1384 patients underwent pancreatic surgery. Hepatobiliary Pancreat Dis Int 2024; 23:331-338. [PMID: 37236826 DOI: 10.1016/j.hbpd.2023.05.004] [Citation(s) in RCA: 8] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2022] [Accepted: 05/09/2023] [Indexed: 05/28/2023]
Abstract
BACKGROUND Pancreatic solid pseudopapillary tumors (SPTs) are rare clinical entity, with low malignancy and still unclear pathogenesis. They account for less than 2% of exocrine pancreatic neoplasms. This study aimed to perform a systematic review of the main clinical, surgical and oncological characteristics of pancreatic SPTs. DATA SOURCES MEDLINE/PubMed, Web of Science and Scopus databases were systematically searched for the main clinical, surgical and oncological characteristics of pancreatic SPTs up to April 2021, in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) standards. Primary endpoints were to analyze treatments and oncological outcomes. RESULTS A total of 823 studies were recorded, 86 studies underwent full-text reviews and 28 met inclusion criteria. Overall, 1384 patients underwent pancreatic surgery. Mean age was 30 years and 1181 patients (85.3%) were female. The most common clinical presentation was non-specific abdominal pain (52.6% of cases). Mean overall survival was 98.1%. Mean recurrence rate was 2.8%. Mean follow-up was 4.2 years. CONCLUSIONS Pancreatic SPTs are rare, and predominantly affect young women with unclear pathogenesis. Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.
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Affiliation(s)
- Gennaro Mazzarella
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy; Department of Surgery "Pietro Valdoni", Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
| | - Edoardo Maria Muttillo
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy; Surgical and Medical Department of Translational Medicine, Sant'Andrea Hospital, Sapienza University of Rome, 00185 Rome, Italy
| | - Diego Coletta
- Department of Surgical Sciences, Umberto I University Hospital, Sapienza University of Rome, 00136 Rome, Italy; Department of General Surgery, Ospedali Riuniti Marche Nord, 61121 Pesaro, Italy
| | - Biagio Picardi
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy
| | - Stefano Rossi
- Division of General and Emergency Surgery, San Filippo Neri Hospital, 00136 Rome, Italy
| | | | - Vito Gomes
- Department of Pathology, San Filippo Neri Hospital, 00136 Rome, Italy
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Melikian R, Kulkarni S. Bifocal solid pseudopapillary tumour of the pancreas. BMJ Case Rep 2024; 17:e259724. [PMID: 38969395 DOI: 10.1136/bcr-2024-259724] [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: 07/07/2024] Open
Abstract
Solid pseudopapillary neoplasm of the pancreas (SPNP) is a rare entity. In this study, we present a woman in her 20's who presented for evaluation of two separate pancreatic masses. On imaging and biopsy, the tail lesion was thought to be a neuroendocrine tumour and the body lesion was thought to be a metastatic lymph node. The patient was brought to the operating room and underwent a distal pancreatectomy and splenectomy. The patient had an uneventful postoperative course and was discharged home on postoperative day 4. Pathology confirmed both masses were consistent with the diagnosis of well-differentiated SPNP with no signs of malignancy including lymphovascular or perineural invasion, or lymph node involvement.
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Affiliation(s)
- Raffi Melikian
- Department of Hepatobiliary and Pancreatic Surgery, Keck Medical Center, Los Angeles, California, USA
| | - Sujit Kulkarni
- General Surgery, Keck Medical Center of USC, Los Angeles, California, USA
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Chen CC, Feng TY, Jan HC, Chou SJ, Chen TH, Wang SC. Rare case of solid pseudopapillary neoplasm of the pancreas with liver and splenic metastasis in a 19-year-old girl. Int J Surg Case Rep 2024; 120:109867. [PMID: 38870658 PMCID: PMC11258626 DOI: 10.1016/j.ijscr.2024.109867] [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: 04/30/2024] [Revised: 05/30/2024] [Accepted: 05/31/2024] [Indexed: 06/15/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare neoplasms, accounting for only 1 %-2 % of all pancreatic tumors, and predominantly affect female patients. CASE PRESENTATION The present case report details a patient presenting to the emergency department with abdominal pain for 3 days who ultimately received a diagnosis of SPNs in the pancreatic body and tail. A contrast-enhanced computed tomography (CT) scan revealed a sizable mass arising from the pancreas, featuring an enhancing cystic component with involvement of the liver and spleen. The patient underwent subsequent exploratory laparotomy, a distal pancreatectomy, splenectomy, and partial hepatectomy. SPN diagnosis was confirmed by histopathology and immunohistochemistry with negative resection margins. CLINICAL DISCUSSION Approximately 70 % of SPN cases are asymptomatic and are incidentally discovered. Despite advances in diagnostic modalities, preoperative diagnosis of SPNs remains a clinical challenge. Surgical management with negative resection margins remains the primary treatment approach. The recurrence rate after surgical resection has been reported to be 3 %-9 %. The prognosis for SPNs limited to the pancreas is generally favorable, with a cure rate exceeding 95 % after complete surgical resection. CONCLUSION An SPN of the pancreas is a rare tumor observed in young female patients. Although it is classified as a malignant tumor, SPN has low malignant potential. Aggressive surgical resection, however, has proven effective in curing SPN for the majority of patients.
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Affiliation(s)
- Chi-Chi Chen
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan
| | - Ting-Yuan Feng
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan
| | - Hsiang-Chun Jan
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan
| | - Shaw-Jiun Chou
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan
| | - Tzu-Hung Chen
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan
| | - Sheng-Chun Wang
- Division of General Surgery, Department of Surgery, Cardinal Tien Hospital, New Taipei, Taiwan.
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Khristenko E, Gaida MM, Tjaden C, Steinle V, Loos M, Krieger K, Weber TF, Kauczor HU, Klauß M, Mayer P. Imaging differentiation of solid pseudopapillary neoplasms and neuroendocrine neoplasms of the pancreas. Eur J Radiol Open 2024; 12:100576. [PMID: 38882634 PMCID: PMC11176946 DOI: 10.1016/j.ejro.2024.100576] [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: 03/22/2024] [Revised: 05/21/2024] [Accepted: 05/29/2024] [Indexed: 06/18/2024] Open
Abstract
Purpose The present study aimed to compare the computed tomography (CT) and magnetic resonance imaging (MRI) features of solid pseudopapillary neoplasms (SPNs) and pancreatic neuroendocrine neoplasms (pNENs). Method Lesion imaging features of 39 patients with SPNs and 127 patients with pNENs were retrospectively extracted from 104 CT and 91 MRI scans. Results Compared to pNEN patients, SPN patients were significantly younger (mean age 51.8 yrs versus 32.7 yrs) and more often female (female: male ratio, 5.50:1 versus 1.19:1). Most SPNs and pNENs presented as well-defined lesions with an expansive growth pattern. SPNs more often appeared as round or ovoid lesions, compared to pNENs which showed a lobulated or irregular shape in more than half of cases (p<0.01). A surrounding capsule was detected in the majority of SPNs, but only in a minority of pNENs (<0.01). Hemorrhage occurred non-significantly more often in SPNs (p=0.09). Signal inhomogeneity in T1-fat-saturated (p<0.01) and T2-weighted imaging (p=0.046) as well as cystic degeneration (p<0.01) were more often observed in SPNs. Hyperenhancement in the arterial and portal-venous phase was more common in pNENs (p<0.01). Enlargement of locoregional lymph nodes (p<0.01) and liver metastases (p=0.03) were observed in some pNEN patients, but not in SPN patients. Multivariate logistic regression identified the presence of a capsule (p<0.01), absence of arterial hyperenhancement (p<0.01), and low patient age (p<0.01), as independent predictors for SPN. Conclusions The present study provides three key features for differentiating SPNs from pNENs extracted from a large patient cohort: presence of a capsule, absence of arterial hyperenhancement, and low patient age.
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Affiliation(s)
- Ekaterina Khristenko
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Matthias M Gaida
- Institute of Pathology, University Medical Center Mainz, JGU-Mainz, Mainz 55131, Germany
- Joint Unit Immunopathology, Institute of Pathology, University Medical Center, JGU-Mainz and TRON, Translational Oncology at the University Medical Center, JGU-Mainz, Mainz 55131, Germany
- Institute of Pathology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Christine Tjaden
- Department of General, Visceral, and Transplantation Surgery, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Verena Steinle
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Martin Loos
- Department of General, Visceral, and Transplantation Surgery, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Korbinian Krieger
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
- Department of Nuclear Medicine, Inselspital, University Hospital Bern, Bern 3010, Switzerland
| | - Tim F Weber
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Hans-Ulrich Kauczor
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Miriam Klauß
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
| | - Philipp Mayer
- Clinic for Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg 69120, Germany
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12
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Lee J, Song SH, Lee IH, Kim DJ, Lee HJ. Primary Solid Pseudopapillary Tumor of the Ovary: A Case Report and Review of the Literature. J Clin Med 2024; 13:2791. [PMID: 38792331 PMCID: PMC11121763 DOI: 10.3390/jcm13102791] [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: 03/25/2024] [Revised: 05/07/2024] [Accepted: 05/08/2024] [Indexed: 05/26/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasms (SPNs) are rare and mainly originate from the pancreas. SPNs originating from the ovary (SPN-O) are extremely rare, and only 13 cases have been reported in the English literature since 2010. CASE We report a 31-year-old woman with SPN-O accompanied by multiple metastases in the abdominal cavity. The patient underwent staging surgery and cytoreduction. Furthermore, the multidisciplinary board decided on adjuvant chemotherapy with an FP regimen (fluorouracil plus cisplatin) because a microscopic metastasis was discovered in the peritoneum near the appendix. Next-generation sequencing showed some pathologic mutations of oncogenes/cancer-associated genes, including CTNNB1 and TP53. This is the fourteenth case of SPN-O and the first one to demonstrate the TP53 pathogenic mutant variant in SPN-O. The patient showed 8 months of disease-free survival until February 2024. CONCLUSION The combination of R0 cytoreduction with FOLFIRI chemotherapy appears to be an effective and feasible treatment option.
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Affiliation(s)
- Juhun Lee
- Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Kyungpook Nation University Hospital, Daegu 41944, Republic of Korea;
| | - Seung Ho Song
- Department of Surgery, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu 41944, Republic of Korea;
| | - In Hee Lee
- Department of Hematology/Oncology, School of Medicine, Kyungpook National University, Kyungpook National University Chilgok Hospital, Daegu 41404, Republic of Korea;
| | - Dong Ja Kim
- Department of Forensic Medicine, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu 41404, Republic of Korea;
| | - Hyun Jung Lee
- Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Kyungpook Nation University Hospital, Daegu 41944, Republic of Korea;
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13
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Hu Y, Jones D, Esnakula AK, Krishna SG, Chen W. Molecular Pathology of Pancreatic Cystic Lesions with a Focus on Malignant Progression. Cancers (Basel) 2024; 16:1183. [PMID: 38539517 PMCID: PMC10969285 DOI: 10.3390/cancers16061183] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2024] [Revised: 03/12/2024] [Accepted: 03/14/2024] [Indexed: 11/11/2024] Open
Abstract
The malignant progression of pancreatic cystic lesions (PCLs) remains understudied with a knowledge gap, yet its exploration is pivotal for effectively stratifying patient risk and detecting cancer at its earliest stages. Within this review, we delve into the latest discoveries on the molecular level, revealing insights into the IPMN molecular landscape and revised progression model, associated histologic subtypes, and the role of inflammation in the pathogenesis and malignant progression of IPMN. Low-grade PCLs, particularly IPMNs, can develop into high-grade lesions or invasive carcinoma, underscoring the need for long-term surveillance of these lesions if they are not resected. Although KRAS and GNAS remain the primary oncogenic drivers of neoplastic development in IPMNs, additional genes that are important in tumorigenesis have been recently identified by whole exome sequencing. A more complete understanding of the genes involved in the molecular progression of IPMN is critical for effective monitoring to minimize the risk of malignant progression. Complicating these strategies, IPMNs are also frequently multifocal and multiclonal, as demonstrated by comparative molecular analysis. Algorithms for preoperative cyst sampling and improved radiomic techniques are emerging to model this spatial and temporal genetic heterogeneity better. Here, we review the molecular pathology of PCLs, focusing on changes associated with malignant progression. Developing models of molecular risk stratification in PCLs which can complement radiologic and clinical features, facilitate the early detection of pancreatic cancer, and enable the development of more personalized surveillance and management strategies are summarized.
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Affiliation(s)
- Yan Hu
- James Molecular Laboratory, The Ohio State University Comprehensive Cancer Center, Columbus, OH 43210, USA; (Y.H.); (D.J.)
| | - Dan Jones
- James Molecular Laboratory, The Ohio State University Comprehensive Cancer Center, Columbus, OH 43210, USA; (Y.H.); (D.J.)
| | - Ashwini K. Esnakula
- Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
| | - Somashekar G. Krishna
- Division of Gastroenterology, Hepatology, and Nutrition, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
| | - Wei Chen
- Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
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14
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Fu C, Li X, Wang Y, Wang C, Jin H, Liu K, Xu H. Solid pseudopapillary neoplasm of the pancreas: a retrospective study of 195 cases. Front Oncol 2024; 14:1349282. [PMID: 38469229 PMCID: PMC10925641 DOI: 10.3389/fonc.2024.1349282] [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: 12/04/2023] [Accepted: 02/12/2024] [Indexed: 03/13/2024] Open
Abstract
Objective Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare exocrine tumor of the pancreas. The aim of our study is to summarize the clinical features of SPN and to analyze the risk factors for malignant SPN. Methods From May 2013 to September 2022, patients who were pathologically confirmed to have SPN were retrospectively reviewed. Demographic data, clinical and pathological features, follow-up data were collected and analyzed. To investigate the factors influencing the benign or malignant nature of SPN, we employed logistic regression. Additionally, we utilized Kaplan-Meier curves to depict and analyze the overall prognosis. Results A total of 195 patients were included, 163 of whom were female and the average age of all patients was 31.7 years old. Among 195 patients, 101 patients (51.8%) had no obvious clinical symptoms and their pancreatic lesions were detected during routine examination. The primary symptom was abdominal pain and distension in 64 cases (32.8%). The maximum diameter of SPN tumors ranged from 1-17 cm (mean 6.19 cm). Forty-eight postoperative complications developed in 43 (22.1%) patients. After a median follow-up duration of 44.5 months, the overall 5-year survival rate was 98.8% and the recurrence rate was 1.5%. Furthermore, we observed a statistically significant difference in the completeness of the tumor capsule between benign and malignant SPN. Conclusion SPN is associated with a favorable long-term survival after surgery in our large sample size cohort. For malignant SPN, tumor capsule incompleteness is an independent risk factor.
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Affiliation(s)
- Chang Fu
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Xiaocong Li
- Medical Research and Biometrics Center, National Clinical Research Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yongxin Wang
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Chuangshi Wang
- Medical Research and Biometrics Center, National Clinical Research Center for Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hengwei Jin
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Kai Liu
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Hongji Xu
- Department of Abdominal Surgery, Guiqian International General Hospital, Guiyang, Guizhou, China
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Guo J, Zhao Q, Qin L, Xie S, Lu S, Li B, He M, Xie L, Yu S. Massive extrapancreatic solid pseudopapillary neoplasm misdiagnosed as hepatic tumor: a case report and literature review. Front Oncol 2024; 14:1342400. [PMID: 38380363 PMCID: PMC10877141 DOI: 10.3389/fonc.2024.1342400] [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: 11/21/2023] [Accepted: 01/16/2024] [Indexed: 02/22/2024] Open
Abstract
Background Solid pseudopapillary neoplasms (SPNs) of the pancreas are uncommon, low-malignancy neoplasms. Moreover, the occurrence of extrapancreatic SPNs is rarely encountered. Case summary A 45-year-old female presented with a right upper abdominal mass and abdominal pain for 3 and 1 months as chief complaints, respectively. Initially, the patient was misdiagnosed with hepatocellular carcinoma based on her symptoms and results of physical and imaging examinations. Following multidisciplinary discussion and ruling out surgical contraindications, a decision was taken to proceed with surgical intervention. Interestingly, the tumor was found to originate from the retroperitoneum and had invaded the right half of the liver and the right wall of the inferior vena cava. The operation was uneventful, and the pathological findings confirmed the tumor as an extrapancreatic SPN. The patient remained asymptomatic after 15 months of follow-up. Conclusion Surgical treatment remains the preferred option for extrapancreatic SPN. The preoperative misdiagnosis also highlights the importance of accurate diagnosis and the development of appropriate treatment strategies for liver masses.
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Affiliation(s)
- Jixu Guo
- Division of Hepatobiliary Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Qingjuan Zhao
- The First Clinical Medical College, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Liting Qin
- Division of Pathology, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Shengjie Xie
- Division of Hepatobiliary Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Shiliu Lu
- Division of Hepatobiliary Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Baibei Li
- Division of Hepatobiliary Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Meilin He
- The First Clinical Medical College, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Linhong Xie
- Division of Hepatobiliary Surgery, The Second People’s Hospital of Qinzhou, Qinzhou, China
| | - Shuiping Yu
- Division of Hepatobiliary Surgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
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Jiménez-Gutiérrez JM, de la Mora-Levy JG, Alonso-Lárraga JO, Hernández-Guerrero AI, Soriano-Herrera BA, Villegas-González LF, Uscanga-Domínguez LF, López-Romero S, Téllez-Ávila FI. EUS-guided tissue acquisition in patients with solid pseudopapillary neoplasms of the pancreas. Postgrad Med 2024; 136:78-83. [PMID: 38315145 DOI: 10.1080/00325481.2024.2313446] [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: 08/12/2023] [Accepted: 01/09/2024] [Indexed: 02/07/2024]
Abstract
OBJECTIVES Endoscopic ultrasound-guided tissue acquisition (EUS-TA) has been the most common method used for the preoperative cytopathological diagnosis of solid tumors of the pancreas. There are only a few reported cases about the role of endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) in the pre-operative diagnosis of solid pseudopapillary neoplasms (SPN). This study aimed to evaluate the diagnostic yield of EUS-TA,including endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) andEUS-FNB, in patients with SPN. METHODS We performed a retrospective analysis of patients with EUS-TA for SPN diagnosis in 2 referral centers. The primary outcome was the diagnostic yield of EUS-TA compared to the surgical specimen. RESULTS Seventy-four patients with SPN of the pancreas were identified. Eighteen had a EUS-TA (10 EUS-FNB and 8 EUS-FNA). The median age of the patients was 31 years (IQR 21-38), and all patients were women. The most common presenting symptom was abdominal pain. Most of the tumors were in the head of the pancreas (9/18; 50%). The median tumor size by EUS was 4.5 cm (min-max 2-15 cm). The most common appearance on EUS was a solid lesion (n = 8/18, 44.4%). A definitive presurgical cytopathological diagnosis was obtained in 16/18 patients (88.8%) with EUS-TA. The sensitivity and positive predictive value of the EUS-TA were 94% each. One patient in the EUS-FNB group developed mild acute pancreatitis. CONCLUSION The diagnostic yield of the EUS-TA in SPN is high. In most cases, the diagnosis was obtained with the first procedure. No differences in the diagnostic yield or AEs between EUS-FNA vs. EUS-FNB needles were seen.
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Affiliation(s)
| | | | - Juan O Alonso-Lárraga
- Department of Endoscopy, Instituto Nacional de Cancerología, Ciudad de México, México
| | | | | | | | - Luis F Uscanga-Domínguez
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, México
| | - Stephanie López-Romero
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, México
| | - Félix I Téllez-Ávila
- Division of Gastroenterology & Hepatology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
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Meliti A, Al-Maghrabi J. Solid Pseudopapillary Neoplasm of the Pancreas: A Clinicopathological Study of 12 Cases With Emphasis on Diagnostic Pitfalls. Cureus 2023; 15:e49858. [PMID: 38169685 PMCID: PMC10758586 DOI: 10.7759/cureus.49858] [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] [Accepted: 12/03/2023] [Indexed: 01/05/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasm of the pancreas (SPNP) is a rare primary neoplasm with distinct clinicopathological features. The tumor most commonly occurs in younger (premenopausal) women and is typified by low malignant potential and an excellent overall prognosis. METHODS A retrospective search over 20 years at two referral tertiary care institutions (King Faisal Hospital and Research Center and King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia) revealed 12 female patients diagnosed with SPNPs. The reslts of ancillary studies performed at the time of diagnosis were also reviewed and placed in the context of current recommendations. RESULTS The clinical and pathological findings were reviewed. All patients were females, aged 18 to 30 years. Eight patients presented with abdominal pain, of which two experienced significant weight loss, and four presented with abdominal mass/discomfort. The tumor size ranged from 1.5 and 15 cm. Two cases were initially diagnosed as neuroendocrine tumors (NETs). One of the cases presented as a multifocal disease. All patients were treated surgically with a follow-up period between one and 11 years. Only one patient presented with peritoneal metastasis after seven years of follow-up, but generally, all are doing well. CONCLUSIONS We have analyzed 12 SPNP cases in our population over 20 years (2001-2021) in this study. In brief, SPNP is a low-grade malignant potential tumor. Even though SPNP is a recognized entity, diagnostic challenges can arise particularly in the setting of limited sampling. Pathologists must be aware of the classic morphological features of SPNP and the characteristic profile of immunohistochemistry and be able to differentiate SPNP from other mimickers, especially well-differentiated NETs of the pancreas, and ultimately to avoid misdiagnosis and unnecessary oncologic treatment. Adequate surgical resection with negative margins is associated with an excellent outcome.
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Affiliation(s)
- Abdelrazak Meliti
- Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Jeddah, SAU
- Pathology, Alfaisal University College of Medicine, Riyadh, SAU
| | - Jaudah Al-Maghrabi
- Department of Pathology, Faculty of Medicine, King Abdulaziz University, Jeddah, SAU
- Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Jeddah, SAU
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Dorwal P, Abou-Seif C, Shamassi M, Mitchell C, Clouston D, Pascoe C, McLaren C, Blecher G. Signet ring stromal tumours of testis: a tale of two cases on a morphological spectrum. Pathology 2023; 55:883-886. [PMID: 37100665 DOI: 10.1016/j.pathol.2023.02.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2022] [Revised: 01/22/2023] [Accepted: 02/13/2023] [Indexed: 04/28/2023]
Affiliation(s)
- Pranav Dorwal
- Monash Health, Clayton, Vic, Australia; Monash University, Clayton, Vic, Australia.
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Veron Sanchez A, Santamaria Guinea N, Cayon Somacarrera S, Bennouna I, Pezzullo M, Bali MA. Rare Solid Pancreatic Lesions on Cross-Sectional Imaging. Diagnostics (Basel) 2023; 13:2719. [PMID: 37627978 PMCID: PMC10453474 DOI: 10.3390/diagnostics13162719] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2023] [Revised: 08/15/2023] [Accepted: 08/18/2023] [Indexed: 08/27/2023] Open
Abstract
Several solid lesions can be found within the pancreas mainly arising from the exocrine and endocrine pancreatic tissue. Among all pancreatic malignancies, the most common subtype is pancreatic ductal adenocarcinoma (PDAC), to a point that pancreatic cancer and PDAC are used interchangeably. But, in addition to PDAC, and to the other most common and well-known solid lesions, either related to benign conditions, such as pancreatitis, or not so benign, such as pancreatic neuroendocrine neoplasms (pNENs), there are solid pancreatic lesions considered rare due to their low incidence. These lesions may originate from a cell line with a differentiation other than exocrine/endocrine, such as from the nerve sheath as for pancreatic schwannoma or from mesenchymal cells as for solitary fibrous tumour. These rare solid pancreatic lesions may show a behaviour that ranges in a benign to highly aggressive malignant spectrum. This review includes cases of an intrapancreatic accessory spleen, pancreatic tuberculosis, solid serous cystadenoma, solid pseudopapillary tumour, pancreatic schwannoma, purely intraductal neuroendocrine tumour, pancreatic fibrous solitary tumour, acinar cell carcinoma, undifferentiated carcinoma with osteoclastic-like giant cells, adenosquamous carcinoma, colloid carcinoma of the pancreas, primary leiomyosarcoma of the pancreas, primary and secondary pancreatic lymphoma and metastases within the pancreas. Therefore, it is important to determine the correct diagnosis to ensure optimal patient management. Because of their rarity, their existence is less well known and, when depicted, in most cases incidentally, the correct diagnosis remains challenging. However, there are some typical imaging features present on cross-sectional imaging modalities that, taken into account with the clinical and biological context, contribute substantially to achieve the correct diagnosis.
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Affiliation(s)
- Ana Veron Sanchez
- Hôpital Universitaire de Bruxelles, Institut Jules Bordet, 1070 Brussels, Belgium; (I.B.)
| | | | | | - Ilias Bennouna
- Hôpital Universitaire de Bruxelles, Institut Jules Bordet, 1070 Brussels, Belgium; (I.B.)
| | - Martina Pezzullo
- Hôpital Universitaire de Bruxelles, Hôpital Erasme, 1070 Brussels, Belgium
| | - Maria Antonietta Bali
- Hôpital Universitaire de Bruxelles, Institut Jules Bordet, 1070 Brussels, Belgium; (I.B.)
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20
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Ishii T, Terasaka T, Nishida K, Wada J. Retroperitoneal Solid Pseudopapillary Tumor Mimicking Adrenal Malignant Tumor in a 67-Year-Old Man. JCEM CASE REPORTS 2023; 1:luad090. [PMID: 37908992 PMCID: PMC10580579 DOI: 10.1210/jcemcr/luad090] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/08/2023] [Indexed: 11/02/2023]
Abstract
Solid pseudopapillary tumor (SPT) is a low-grade malignant tumor of the pancreas. SPT typically affects women and can occur in ectopic pancreatic region; however, it also occurs rarely in retroperitoneum. The tumor may be bulky at the time of diagnosis since there is no specific clinical manifestation. Here we present an older male case with retroperitoneal SPT. A 67-year-old man consulted for intermittent fever and lumbago. His basal hormonal profile screened out a functional tumor. Computed tomography (CT) showed a gigantic mass in his left adrenal region. A normal left adrenal gland was not identified, and the tumor's feeding artery was recognized as the left adrenal artery by the contrast-enhanced CT. Adrenal malignant tumor was suspected, and tumor resection was performed. The resected tumor size was 15 × 10 × 9 cm. Histologically, epithelial-like cells with round nuclei and a small amount of eosinophilic cytoplasm proliferated in papillary (around the blood vessels) or uniformly solid form. By immunostaining, tumor cells were vimentin, CD56, cytokeratin AE1/AE3, CD10, β-catenin in the nucleus, cyclin D1, and PgR positive. These findings led to the diagnosis of SPT. Although rare, SPT should be considered as a differential diagnosis in cases of a mass arising from the adrenal region.
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Affiliation(s)
- Takahiro Ishii
- Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, 700-8558, Japan
- Endocrine Center, Okayama University Hospital, Okayama, 700-8558, Japan
| | - Tomohiro Terasaka
- Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, 700-8558, Japan
- Endocrine Center, Okayama University Hospital, Okayama, 700-8558, Japan
| | - Kenji Nishida
- Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, 700-8558, Japan
| | - Jun Wada
- Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, 700-8558, Japan
- Endocrine Center, Okayama University Hospital, Okayama, 700-8558, Japan
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The Role of MRI in the Diagnosis of Solid Pseudopapillary Neoplasm of the Pancreas and Its Mimickers: A Case-Based Review with Emphasis on Differential Diagnosis. Diagnostics (Basel) 2023; 13:diagnostics13061074. [PMID: 36980388 PMCID: PMC10046973 DOI: 10.3390/diagnostics13061074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2023] [Revised: 03/05/2023] [Accepted: 03/09/2023] [Indexed: 03/15/2023] Open
Abstract
Solid pseudopapillary neoplasm (SPN) is rare pancreatic tumor occurring most commonly in young females. The typical imaging appearance of SPN is of well-defined, encapsulated, and large heterogeneous tumors, consisting of solid and cystic components due to various degrees of intralesional hemorrhage and necrosis. However, atypical imaging presentation in the form of small solid tumors or uniformly cystic lesions might also be seen, which can be explained by specific pathological characteristics. Other imaging features such as a round shape, the absence of main pancreatic duct dilatation, and slow growth, in combination with vague symptoms, favor the diagnosis of SPNs. Nevertheless, the radiological findings of SPN might overlap with other solid and cystic pancreatic neoplasms, such as neuroendocrine tumors, serous and mucinous neoplasms, and even small pancreatic adenocarcinomas. In addition, a few benign non-tumorous conditions including walled-of-necrosis, and intrapancreatic accessory spleen may also pose diagnostic dilemmas simulating SPNs on imaging studies. The aim of this manuscript is to provide a comprehensive overview of the typical and atypical imaging features of SPNs and to describe useful tips for differential diagnosis with its potential mimickers.
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22
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Sun G, Fang K, Fu X, Peng L, Shu J, Tu Y, Li Y, Xiao W. Solid Pseudopapillary Neoplasm of the Pancreas: A Multi-Institution Study of 118 Cases. Pancreas 2023; 52:e121-e126. [PMID: 37523603 DOI: 10.1097/mpa.0000000000002219] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 08/02/2023]
Abstract
OBJECTIVES The aim of the study is to summary the clinicopathological characteristics and surgical outcomes of solid pseudopapillary neoplasm (SPN) of the pancreas. METHODS In this retrospective study, the information of 118 patients with SPN from 3 hospitals were analyzed. RESULTS A total of 118 patients. The mean age was 30.8 (standard deviation, 14.7) years and the majority were female (n = 95, 80.5%). Sixty-seven patients (56.8%) had clinical symptoms, of which the most common symptom was abdominal pain (49.6%). The mean tumor size was 5.9 (standard deviation, 2.9) cm. Pseudopapillary architecture was the commonest histologic feature, and β-catenin, CD56, vimentin, neuron-specific enolase, CD10, a1-antitrypsin, cytokeratins showed different degrees of positive expression in immunohistochemical staining. Fourteen patients (11.9%) presented aggressive pathologic behavior, which was correlated to the incomplete tumor capsule. At a median follow-up of 59.2 months, the recurrence rate was 1.8% and the overall 5-year survival rate was 97.7%. CONCLUSIONS Solid pseudopapillary neoplasm of the pancreas is a potentially low-grade malignant tumor that most frequently found in young females. Its clinical manifestations are nonspecific and the diagnosis mostly depends on pathological examination. Surgical resection is the first choice of treatment for SPN with a good prognosis.
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Affiliation(s)
- Gen Sun
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Kang Fang
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Xiaowei Fu
- Department of General Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Long Peng
- Department of General Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Jiaming Shu
- Department of Oncology, Jiangxi Provincial People's Hospital, Nanchang, China
| | - Yi Tu
- Department of Pathology, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Yong Li
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Weidong Xiao
- From the Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
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23
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Meng L, Zhan Y, Wei M, Yang R, Wang J, Weng S, Chen L, Zheng S, Dong K, Dong R. Single-cell RNA sequencing of solid pseudopapillary neoplasms of the pancreas in children. Cancer Sci 2023; 114:1986-2000. [PMID: 36721980 PMCID: PMC10154873 DOI: 10.1111/cas.15744] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2022] [Revised: 01/05/2023] [Accepted: 01/23/2023] [Indexed: 02/02/2023] Open
Abstract
Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor in children. Its origin remains elusive, along with its pathogenesis. Heterogeneity within SPN has not been previously described. In addition, low malignant but recurrent cases have occasionally been reported. To comprehensively unravel these profiles, single-cell RNA sequencing was performed using surgical specimens. We identified the cell types and suggested the origin of pancreatic endocrine progenitors. The Wnt/β-catenin pathway may be involved in tumorigenesis, while the epithelial-to-mesenchymal transition may be responsible for SPN recurrence. Furthermore, NOV, DCN were nominated as primary and S100A10, MGP as recurrent SPN marker genes, respectively. Our results provide insight into the pathogenesis of SPN.
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Affiliation(s)
- Lingdu Meng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Yong Zhan
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Meng Wei
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China.,Department of Hematology, Shanghai Children's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Ran Yang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Junfeng Wang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Shuting Weng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Lian Chen
- Department of Pathology, Children's Hospital of Fudan University, Shanghai, China
| | - Shan Zheng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Kuiran Dong
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
| | - Rui Dong
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, Shanghai, China
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Chen W, Ahmed N, Krishna SG. Pancreatic Cystic Lesions: A Focused Review on Cyst Clinicopathological Features and Advanced Diagnostics. Diagnostics (Basel) 2022; 13:65. [PMID: 36611356 PMCID: PMC9818257 DOI: 10.3390/diagnostics13010065] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Revised: 12/13/2022] [Accepted: 12/19/2022] [Indexed: 12/28/2022] Open
Abstract
Macroscopic, endomicroscopic, and histologic findings and correlation are an integral part of the diagnostic evaluation of pancreatic cystic lesions (PCLs), as complementing morphologic features seen by different specialties are combined to contribute to a final diagnosis. However, malignancy risk stratification of PCLs with worrisome features can still be challenging even after endoscopic ultrasound guided-fine needle aspiration (EUS-FNA) with cytological evaluation. This review aims to summarize cyst clinicopathological features from the pathologists' perspective, coupled with knowledge from advanced diagnostics-confocal laser endomicroscopy and cyst fluid molecular analysis, to demonstrate the state-of-art risk stratification of PCLs. This review includes illustrative photos of surgical specimens, endomicroscopic and histologic images, and a summary of cyst fluid molecular markers.
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Affiliation(s)
- Wei Chen
- Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
| | - Nehaal Ahmed
- School of Medicine, The Ohio State University, Columbus, OH 43210, USA
| | - Somashekar G. Krishna
- Division of Gastroenterology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
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25
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Bansal LK, Kapur N, Gupta AK, Nagpal A, Chaudhary P. Solid Pseudopapillary Neoplasm - Case Series and Review of Literature. Indian J Surg Oncol 2022; 13:765-775. [PMID: 36687233 PMCID: PMC9845477 DOI: 10.1007/s13193-022-01560-4] [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: 01/09/2022] [Accepted: 05/24/2022] [Indexed: 01/25/2023] Open
Abstract
Solid-pseudopapillary neoplasm (SPN) is a variety of solid and cystic tumors of the pancreas. It was first described by Frantz in 1959. It is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, which accounts for about 0.17 to 2.7% of all pancreatic tumors. Here, we are describing 5 cases of pancreatic solid pseudopapillary neoplasm, out of 180 pancreatic tumors, operated in our institution in the 5-year period (2015-2020). Also, we have reviewed all available case series (from 2006 to 2020) in the literature, of pancreatic pseudopapillary neoplasm, for demographic information, etiopathogenesis, diagnosis, and extent of operation to establish the optimal management of this condition. Retrospective analysis of pancreatic tumors was carried out from February 2015 to January 2020. A total of 180 patients underwent pancreatic resection in this period for pancreatic tumor, out of which, the solid pseudopapillary neoplasm was confirmed in 5 cases (2.76%). Among these 5 cases, 4 cases (80%) were female and one (20%) male, with age group range from 14 to 45 years (mean age - 28 years). Abdominal pain was the most frequent presenting symptom (60%). Mean tumor diameter was 6.9 cm (range, 2-18 cm). Two patients were diagnosed preoperatively by CECT and MRI findings, and three patients were diagnosed preoperatively by percutaneous/USG-guided and CT-guided FNA cytology. Two patients underwent pancreatoduodenectomy; one patient underwent enucleation; and two patients underwent spleen preserving distal pancreatectomy. Four patients are alive and on regular follow-up, while one patient died on the 5th post-operative day due to post-operative sepsis.
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Affiliation(s)
- Lalit Kumar Bansal
- Department of Surgery, ABVIMS and Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
| | - Neeti Kapur
- Department of Surgery, ABVIMS and Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
| | - Arun Kumar Gupta
- Department of Surgery, ABVIMS and Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
| | - Ashutosh Nagpal
- Department of Surgery, ABVIMS and Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
| | - Poras Chaudhary
- Department of Surgery, ABVIMS and Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
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Liu H, Xu Z, Wang Y, Gu H, Tang Y, Wu D, Wang J, Zhang J. Case report: A case report and literature review of extrapancreatic solid pseudopapillary neoplasm. Front Surg 2022; 9:1020044. [PMID: 36406380 PMCID: PMC9672344 DOI: 10.3389/fsurg.2022.1020044] [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: 08/15/2022] [Accepted: 10/03/2022] [Indexed: 11/06/2022] Open
Abstract
Background Solid pseudopapillary neoplasm (SPN) is a rare tumor with low malignant potential, which typically occurs in the pancreas. Extrapancreatic SPN is also extremely rare worldwide. Case presentation We report a case of a 70-year-old woman hospitalized with abdominal pain and bloating. The patient did not have any underlying diseases, such as diabetes, coronary heart disease, or hypertension. More than 30 years ago, the patient underwent surgery for “ectopic pregnancy”. The patient had no family history of hereditary disease, nor did any immediate family members have a history of cancer. Laboratory tests showed that her hemoglobin and albumin levels were low and she had a high level of cancer antigen 125 (CA125). Enhanced computed tomography (CT) showed a large tumor in the abdomen and pelvis. The patient subsequently underwent surgery, and it was found that the tumor was attached to the terminal ileum. Pathological findings suggested that the tumor was an extrapancreatic SPN, with an ectopic pancreas found in the tumor tissue. The patient did not receive chemotherapy or radiotherapy after surgery. After 13 months of follow-up, the patient was admitted again with abdominal pain. CT showed tumor recurrence with extensive systemic metastases. The patient and her family refused reoperation and biopsy, and the patient was discharged after the abdominal pain and anemia resolved. Conclusion We report a rare case of extrapancreatic SPN of ileal origin, which could be the first report worldwide. It had aggressive biological features, with recurrence and metastasis 13 months after surgery. For extrapancreatic SPN, the risk of recurrence should be assessed, and for tumors suspected of malignant behavior, a longer follow-up after discharge may be needed. Although SPN generally has a good prognosis after surgery, there is no consensus on whether postoperative chemotherapy and other treatments are needed for patients with high recurrence risk.
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Frías-Fernández P, Sánchez-Flores S, García-Chávez JP, Padilla-Rosciano AE, Lino-Silva LS. Solid and papillary neoplasm of the pancreas (SPNP): a case report. AME Case Rep 2022; 6:38. [PMID: 36339914 PMCID: PMC9634458 DOI: 10.21037/acr-22-30] [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: 06/06/2022] [Accepted: 10/09/2022] [Indexed: 11/07/2022]
Abstract
BACKGROUND Solid and papillary neoplasm of the pancreas (SPNP) is a rare pancreatic tumor, well known for its predilection for young women and large volume. The tumor has a favorable prognosis and differentiating it from other pancreatic tumors with aggressive behavior is necessary. CASE DESCRIPTION We present the case of a 34-year-old female without relevant background. She presented with abdominal pain and by fine needle biopsy was diagnosed as ductal carcinoma. During the evaluation, an abdominal ultrasound revealed a pancreatic growth that was "bulky, solid, with irregular margins, in homogeneously hypoechoic, with anechoic areas of necrosis, located lateral to the tail of the pancreas and medial to the upper pole of the left kidney and the lower splenic pole". The patient was admitted, and surgery was performed. At the laparotomy, a tumor of 15 cm in diameter was detected. The tumor was located in the tail of the pancreas, was well encapsulated, and of solid consistency. Caudal pancreatectomy with a splenectomy was carried out. The final pathology diagnosis was a SPNP. CONCLUSIONS In the presence of a large abdominal mass of pancreatic relevance, even in older women, the possibility of having an SPNP should always be evaluated. Given the low malignancy potential of this tumor and the excellent prognosis with radical surgical treatment, the preoperative diagnosis should always be particularly accurate. Surgical resection is recommended as the treatment of choice.
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Affiliation(s)
- Pedro Frías-Fernández
- Department of Surgical Oncology, Oncology Center of Tula’s General Hospital, Tula de Allende, Hidalgo, Mexico
| | - Silviana Sánchez-Flores
- Department of Surgical Oncology, Oncology Center of Tula’s General Hospital, Tula de Allende, Hidalgo, Mexico
| | - Juan P. García-Chávez
- Department of Anesthesia, Oncology Center of Tula’s General Hospital, Tula de Allende, Hidalgo, Mexico
| | | | - Leonardo S. Lino-Silva
- Department of Surgical Pathology, Oncology Center of Tula’s General Hospital, Tula de Allende, Hidalgo, Mexico
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Muacevic A, Adler JR. Solid Pseudopapillary Epithelial Neoplasm of the Pancreas in the Paediatric Population: A Report of Two Cases. Cureus 2022; 14:e29805. [PMID: 36337824 PMCID: PMC9620738 DOI: 10.7759/cureus.29805] [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] [Accepted: 09/30/2022] [Indexed: 11/05/2022] Open
Abstract
A rare pathology, the solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas accounts for approximately 1% of pancreatic neoplasms. Initially called ‘Frantz’s tumour’, it has now been renamed to SPEN by the World Health Organization (WHO). This tumour has a predilection for females and a good prognosis with surgical excision being the treatment of choice. Palpable abdominal masses in children are of significant clinical importance. Identifying cystic lesions in the pancreas from CT or MRI scans always warrant further investigations. Primary pancreatic neoplasms account for 0.1% of pancreatic tumours in the paediatric population; an extremely rare circumstance constituting a diagnostic and therapeutic challenge to surgeons. This article comprises two paediatric cases of SPEN in 14- and 11-year-old females, respectively, and a literature review on current management.
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Pregnancy Complicated With a Giant Pancreatic Tumor and Decompensation of Liver Cirrhosis: A Case Report and Literature Review. MATERNAL-FETAL MEDICINE 2022. [DOI: 10.1097/fm9.0000000000000168] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
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Dhali A, Ray S, Das S, Mandal TS, Khamrui S, Gupta A, Ansari Z, Ghosh R, Dhali GK. Clinical profile and outcome of solid pseudopapillary neoplasm of the pancreas: A retrospective study on 28 patients. Int J Surg 2022; 104:106708. [PMID: 35700958 DOI: 10.1016/j.ijsu.2022.106708] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2022] [Revised: 05/24/2022] [Accepted: 05/26/2022] [Indexed: 11/17/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasms (SPN) of the pancreas are rare tumors accounting for 0.9-2.7% of all exocrine pancreatic tumors. Very few studies comprising of more than 10 patients have been published. The aim of the present study is to report on our experience with SPNs over a period of 14 years from a tertiary center of Eastern India. METHODOLOGY Data of all patients whose histopathology reports of surgically resected specimen confirmed SPN were retrospectively reviewed in the present study. RESULTS Twenty-eight patients had a pathologically confirmed diagnosis of SPN. Twenty-five (89.3%) patients were females with a median age of 26 (15-45) years. Abdominal pain (89.2%) was the most common presenting symptom. Abdominal mass was palpable in 12 (42.8%) patients. The mean size of the tumor was 9.03 cm (range, 4-25 cm). The most common location of the tumor was in the body and tail of pancreas (35.7%). The most commonly performed operation was distal pancreaticosplenectomy (n = 17, 60.7%), followed by Whipple's procedure (n = 8, 28.5%). Thirty postoperative complications developed in 23 (82.1%) patients. The operative mortality was 3.5% (n = 1). The median hospital stay was 10 (5-25) days. Over a median follow-up period of 36 months (range), no patient developed recurrence. CONCLUSION Although the size of tumor was quite large at the time of initial presentation, complete surgical resection was possible in all the patients. In experienced hand, surgery can be performed with acceptable perioperative mortality and excellent long-term outcomes.
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Affiliation(s)
- Arkadeep Dhali
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Sukanta Ray
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India.
| | - Somak Das
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Tuhin Subhra Mandal
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Sujan Khamrui
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Arunesh Gupta
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Zuber Ansari
- Department of GI Surgery, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Ranajoy Ghosh
- Department of GI Pathology, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
| | - Gopal Krishna Dhali
- Department of Gastroenterology, IPGME&R, School of Digestive & Liver Diseases, Kolkata, India
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Biswas J, Karpha K, Nath S, Dhali A, Dhali GK. Solid Pseudopapillary Neoplasm of the Pancreas Presenting With Gastric Outlet Obstruction. Cureus 2022; 14:e24092. [PMID: 35573581 PMCID: PMC9106554 DOI: 10.7759/cureus.24092] [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] [Accepted: 04/13/2022] [Indexed: 11/20/2022] Open
Abstract
Solid pseudopapillary neoplasm (SPN) of the pancreas are rare tumors accounting for a minor portion of all exocrine pancreatic tumors. It usually occurs in young women. It has a very low malignant potential with a relatively indolent clinical course. A small subset of patients exhibits pathological features of malignancy. Herein, we present a rare case of pancreatic SPN that presented with gastric outlet obstruction. Despite the characteristic computed tomography (CT) findings, due to its rarity, it was missed in more common conditions such as gastrointestinal stromal tumors. In our case, we found that the tumor was causing extrinsic duodenal compression leading to gastric outlet obstruction, creating a diagnostic dilemma.
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Affiliation(s)
- Jyotirmoy Biswas
- Medicine, College of Medicine and Sagore Dutta Hospital, Kolkata, IND
| | - Kankana Karpha
- Medicine, College of Medicine and Sagore Dutta Hospital, Kolkata, IND
| | - Siddhartha Nath
- Medicine, College of Medicine and Sagore Dutta Hospital, Kolkata, IND
| | - Arkadeep Dhali
- Gastrointestinal Surgery, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, Kolkata, IND
| | - Gopal Krishna Dhali
- Gastroenterology, School of Digestive and Liver Diseases, Institute of Postgraduate Medical Education and Research, Kolkata, IND
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Parra-Herran C, McCluggage WG. Ovarian microcystic stromal tumour: From morphological observations to syndromic associations. Histopathology 2022; 80:898-904. [PMID: 35020947 DOI: 10.1111/his.14616] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2021] [Revised: 12/15/2021] [Accepted: 01/10/2022] [Indexed: 11/30/2022]
Abstract
Microcystic stromal tumour (MST) is a rare, usually benign, ovarian neoplasm characterised morphologically in its classic form by a distinctive triad of features comprising microcysts, solid cellular regions and fibrous stroma. Variant morphology also occurs, including the presence of nests, tubules, cords, and signet ring cells. Immunohistochemically, this neoplasm is characterised by diffuse nuclear expression of β-catenin, cyclin D1, WT1 and SF1, as well as diffuse staining with FOXL2 and CD10. Inhibin and calretinin are typically negative. At the genomic level, these neoplasms harbour mutually exclusive mutations in CTNNB1 or APC genes with the former being significantly more common. This molecular characteristic raises possible links to other rare ovarian lesions including solid pseudopapillary tumour, signet-ring stromal tumour and Sertoli cell tumour. Rarely, MST is an extracolonic manifestation of familial adenomatous polyposis (FAP) and serves as a sentinel event that could trigger the identification of the syndrome. Herein, we review the published literature on ovarian MST and provide practical advice for pathologists reporting these rare neoplasms.
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Affiliation(s)
- Carlos Parra-Herran
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
| | - W Glenn McCluggage
- Department of Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, United Kingdom
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Wang X, Zhu D, Bao W, Li M, Wang S, Shen R. Prognostic Enigma of Pancreatic Solid Pseudopapillary Neoplasm: A Single-Center Experience of 63 Patients. Front Surg 2021; 8:771587. [PMID: 34881287 PMCID: PMC8645639 DOI: 10.3389/fsurg.2021.771587] [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: 09/06/2021] [Accepted: 10/20/2021] [Indexed: 11/29/2022] Open
Abstract
Background: Studies investigating prognostic factors of solid pseudopapillary neoplasm (SPN) have been published with conflicting findings. Methods: Retrospective analysis of 63 consecutive cases of SPN in our institution from January 2010 to December 2019 was carried out. The clinicopathological features, treatment practices along with survival associations were collected and analyzed. Results: Fifteen patients (23.8%) were male, and 48 (76.2%) were female, with a median age of 34.0 ± 14.5 years. The larger tumor size was correlated with the more mixed components (p = 0.000) and the higher Ki-67 index (p = 0.042). No recurrence was found in the nine patients whose tumors fulfilled the WHO criteria for malignancy due to the presence of at least perineural invasion (6.4%), angiovascular invasion (2.3%), and/or adjacent organ invasion (6.4%). Microscopic infiltrative growth was detected in 9 (14.3%) tumors, which was correlated significantly with the WHO criteria (p = 0.002), capsule invasion (p = 0.005), and pancreatic parenchyma invasion (p = 0.001), but not with disease-free survival (p = 0.13). CD99 was found to be positively expressed in 88.9% (40/45) of tumors and more likely to have depressed Ki-67 index (p = 0.016). After a median follow-up of 58 months, only two patients (3.2%) had a recurrence after their first operation outside of our hospital. No patient died due to tumor progression. Conclusions: Although survival is favorable with aggressive surgery, it is actually difficult to assess the prognostic factors of resected SPNs. Future investigations into the role of clinicopathological evaluation will unveil the prognostic enigma of pancreatic SPN after resection.
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Affiliation(s)
- Xinbo Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Daojun Zhu
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Wei Bao
- Department of Pathology, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Min Li
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Sizhen Wang
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
| | - Rongxi Shen
- Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China
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Molecular Alterations in Solid Pseudopapillary Neoplasm of the Pancreas: The Achilles Heel in Conquering Pancreatic Tumorigenesis. Pancreas 2021; 50:1343-1347. [PMID: 35041331 DOI: 10.1097/mpa.0000000000001928] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Solid pseudopapillary neoplasms of the pancreas are overwhelmingly benign tumors predominately observed in women in the third decade of life. However, their malignant potential, based on local recurrences and metastases, has brought into question the available evidence on their biological behavior. Solid pseudopapillary neoplasms have distanced themselves from other pancreatic tumors with varying morphological appearance, immune profile, and histogenesis. In review of the literature, PubMed was queried using search strings, including "solid pseudopapillary neoplasm" and "molecular," and "immunohistochemistry." Alternative searches were also conducted given the variability in tumor name, including "solid pseudopapillary tumor" and "Frantz tumor." This article provides an in-depth review into the molecular pathways that contribute to the pathogenesis of solid pseudopapillary neoplasms. It also discusses the implications of existing molecular pathways toward tumor aggressiveness and recurrence potential.
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Konukiewitz B, Jesinghaus M, Kasajima A, Klöppel G. Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls. Virchows Arch 2021; 480:247-257. [PMID: 34647171 PMCID: PMC8986719 DOI: 10.1007/s00428-021-03211-5] [Citation(s) in RCA: 31] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2021] [Revised: 09/06/2021] [Accepted: 09/21/2021] [Indexed: 12/29/2022]
Abstract
Common to neuroendocrine neoplasms of the pancreas is their expression of synaptophysin, chromogranin A, and/or INSM1. They differ, however, in their histological differentiation and molecular profile. Three groups can be distinguished: well-differentiated neuroendocrine neoplasms (neuroendocrine tumors), poorly differentiated neuroendocrine neoplasms (neuroendocrine carcinomas), and mixed neuroendocrine-non-neuroendocrine neoplasms. However, the expression of synaptophysin and, to a lesser extent, also chromogranin A is not restricted to the neuroendocrine neoplasms, but may also be in a subset of non-neuroendocrine epithelial and non-epithelial neoplasms. This review provides the essential criteria for the diagnosis of pancreatic neuroendocrine neoplasms including diagnostic clues for the distinction of high-grade neuroendocrine tumors from neuroendocrine carcinomas and an algorithm avoiding diagnostic pitfalls in the delineation of non-neuroendocrine neoplasms with neuroendocrine features from pancreatic neuroendocrine neoplasms.
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Affiliation(s)
- Björn Konukiewitz
- Institute of Pathology, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Christian-Albrechts-Universität zu Kiel, Arnold-Heller-Straße 3/14, 24105, Kiel, Germany.
| | - Moritz Jesinghaus
- Institute of Pathology, Universitätsklinikum Marburg, Baldingerstraße, 35043, Marburg, Germany
| | - Atsuko Kasajima
- Institute of Pathology, Technische Universität München, Trogerstraße 18, 81675, Munich, Germany
| | - Günter Klöppel
- Institute of Pathology, Technische Universität München, Trogerstraße 18, 81675, Munich, Germany
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Rare Pseudopapillary Neoplasm of the Pancreas: A 10-Year Experience. Surg Res Pract 2021; 2021:7377991. [PMID: 34568545 PMCID: PMC8463183 DOI: 10.1155/2021/7377991] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Revised: 09/06/2021] [Accepted: 09/08/2021] [Indexed: 01/04/2023] Open
Abstract
Introduction The solid pseudopapillary epithelial neoplasm (SPN) is a rare form of pancreatic neoplasm with an incidence of 2-3% of all pancreatic tumours. The recent increase in incidence is attributed to the increasing use of imaging techniques for nonspecific abdominal complaints. We report our institutional experience in the management of this tumour over the last decade. Method We retrospectively analyzed from a prospectively maintained database of patients from January 2011 to December 2020 who were operated upon for SPN. All the patients were followed till date. Results Of 479 patients operated on for various types of pancreatic tumours during this period, 15 (3.1%) had SPN. The mean age of presentation was 28 years with a female preponderance (12/15, 80%). The most common location was the body and tail of the pancreas (66%), and the mean size was 6.4 cm (2–15 cm). The tumour extent was defined as ‘borderline resectable' in 20% of cases. Distal pancreatectomy was done in 11 patients with spleen preservation in 3. R0, R1, and R2 resection were done in 12, 2, and 1 patient(s), respectively. The operative mortality was 6.7%. All the patients are doing well on follow-up. Conclusion SPN is a low-grade malignant tumour with a strong female predilection. Clinical manifestations have no specificity, imaging examination only contributes tumour location, and the final diagnosis rests on pathology. Surgery is the main modality of treatment and carries a good prognosis.
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Lymphoid Enhancer Binding Factor 1 (LEF1) and Paired Box Gene 8 (PAX8): A Limited Immunohistochemistry Panel to Distinguish Solid Pseudopapillary Neoplasms and Pancreatic Neuroendocrine Tumors. Appl Immunohistochem Mol Morphol 2021; 28:776-780. [PMID: 32723981 DOI: 10.1097/pai.0000000000000830] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Solid pseudopapillary neoplasms (SPNs) and pancreatic neuroendocrine tumors (PanNETs) are distinctive entities. However, due to overlapping morphologies, distinguishing them remains a diagnostic challenge. Our study investigates the utility of immunohistochemistry for nuclear lymphoid enhancer binding factor 1 (LEF1) and paired box gene 8 (PAX8) in differentiating these 2 entities. LEF1 and PAX8 immunohistochemistry were performed on fine-needle aspiration cell blocks and surgical resection specimens diagnosed as SPN or PanNET at our institution from January 2007 to August 2016. Eight SPN and 25 PanNET cell blocks and 17 SPN and 34 PanNET surgical resection specimens were examined. On cell blocks, positive staining for LEF1 had high frequency, sensitivity, and specificity for SPN (88%, 88%, and 88%) as did positive staining for PAX8 for PanNET (76%, 76%, and 75%). The findings on surgical resection specimens were consistent with those from cell blocks (LEF1+ in SPN: 100%, 100%, and 97%; PAX8+ in PanNET: 59%, 59%, and 100%). A combined LEF1+/PAX8- phenotype showed high sensitivity and specificity for SPN (cell block: 63% and 100%; surgical specimen: 100% and 98%) as did a LEF1-/PAX8+ phenotype for PanNET (cell block: 64% and 100%; surgical specimen: 59% and 100%). SPN and PanNET exhibit opposite immunophenotypic profiles with LEF1+/PAX8- in SPN and LEF1-/PAX8+ in PanNET. The combination of these 2 stains provides an effective means of distinguishing these 2 entities.
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Silano F, de Melo Amaral RB, Santana RC, Neves VC, Ardengh JC, do Amaral PCG. Yield of surgery in solid pseudopapillary neoplasms of the pancreas: A case series and literature review. World J Gastrointest Oncol 2021; 13:589-599. [PMID: 34163575 PMCID: PMC8204350 DOI: 10.4251/wjgo.v13.i6.589] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/03/2020] [Revised: 03/24/2021] [Accepted: 05/07/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasms (SPN) of the pancreas represents approximately 2% of non-endocrine tumors of the pancreas. It is described in the literature as a rare and predominant tumor in young women. AIM To report a case series with SPN and analyzing clinical, surgical, anatomopathological characteristics, as well as the prognosis and review of literature. METHODS Retrospective analysis of patients undergoing surgery, with histological diagnosis of SPN between 1998 and 2018, using standardized and prospectively completed forms, performed at the Surgery Service of the Upper Digestive System at Hospital São Rafael/Rede D'Or in Salvador - BA. Review of literature through a database search in MEDLINE/PubMed of retrospective articles. RESULTS Fourteen female patients with the average age of 31.6 years (range min-max) were selected. Twelve patients (85.7%) were asymptomatic, being an incidental diagnosis or due to screening for other reasons. One patient had abdominal pain due to gastric compression and another patient had jaundice. The 14 patients were staged with computerized tomography or magnetic resonance imaging. None had evidence of metastasis. In 8 patients (57.1%), the tumor was in the tail and body. The average size was 6.7 cm (range min-18). The type of surgery was according to the anatomical location of the tumor. There was no lymph node involvement. In two cases, vascular resection with the use of a prosthesis was required for reconstruction. The surgical margins were free. In all cases, postoperative immunohistochemistry confirmed that it was a solid pseudo-papillary neoplasia of the pancreas. There has been no disease recurrence in any case so far. CONCLUSION The tumors had a benign, indolent and histopathological behavior compatible with the literature. Curative surgery is recommended in all cases.
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Affiliation(s)
- Flávio Silano
- Digestive Surgery, Hospital São Rafael/Rede D’Or, Salvador 40285000, Bahia, Brazil
| | - Ricardo Bandeira de Melo Amaral
- Department of Surgery of the Upper Digestive System, São Rafael Hospital/Rede D’Or Hospital Group, Salvador 41253-190, Bahia, Brazil
| | - Rodolfo Carvalho Santana
- Department of Surgery of the Upper Digestive System, São Rafael Hospital/Rede D’Or Hospital Group, Salvador 41253-190, Bahia, Brazil
| | - Vanessa Costa Neves
- Department of Surgery of the Upper Digestive System, São Rafael Hospital/Rede D’Or Hospital Group, Salvador 41253-190, Bahia, Brazil
| | - José Celso Ardengh
- Surgery and Anatomy, Hospital das Clinicas da Faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo, São Paulo 04611-000, São Paulo, Brazil
- Endoscopy Service, Hospital 9 de Julho, São Paulo 04611-000, São Paulo, Brazil
- Imaging and Diagnosis, Escola Paulista de Medicina - São Paulo Federal University, São Paulo 04611-000, SP, Brazil
| | - Paulo Cezar Galvão do Amaral
- Department of Surgery of the Upper Digestive System, São Rafael Hospital/Rede D’Or Hospital Group, Salvador 41253-190, Bahia, Brazil
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Guo W, Farzaneh T, Lee W, Nael A, Li X, Chandan VS. A limited panel of INSM1 and LEF1 immunostains accurately distinguishes between pancreatic neuroendocrine tumor and solid pseudopapillary neoplasm. Pathol Res Pract 2021; 223:153462. [PMID: 34048981 DOI: 10.1016/j.prp.2021.153462] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/10/2021] [Revised: 05/01/2021] [Accepted: 05/02/2021] [Indexed: 02/07/2023]
Abstract
Solid pseudopapillary neoplasm (SPN) and well differentiated pancreatic neuroendocrine tumor (PNET) can show significant cytomorphological overlap. In this study, we evaluated the role of INSM1 and LEF1 immunohistochemical stains in distinguishing between these two tumors. 22 SPN and 25 PNET surgically resected cases were stained for both INSM1 and LEF1. All the 22 cases of SPN showed strong and diffuse nuclear staining for LEF1 (in >95 % of tumor cells), while all 25 PNET were negative for LEF1. All 25 PNET cases were positive for INSM1 (moderate to strong intensity nuclear staining in >50 % of the tumor cells), while all 22 cases of SPN were negative for INSM1. The results of our study show that a limited panel comprising of INSM1 and LEF1 immunostains accurately distinguishes between SPN and PNET.
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Affiliation(s)
- Wenchang Guo
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States
| | - Ted Farzaneh
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States
| | - Whayoung Lee
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States
| | - Ali Nael
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States; Department of Pathology, Children Hospital of Orange County (CHOC), Orange, CA, United States
| | - Xiaodong Li
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States
| | - Vishal S Chandan
- Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, United States.
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Michael S, Kassam NM, Njau A, Sherman OA, Chuwa H, Surani S. A Rare Case of Solid Pseudopapillary Neoplasm of the Pancreas. Cureus 2021; 13:e14720. [PMID: 34079673 PMCID: PMC8159312 DOI: 10.7759/cureus.14720] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023] Open
Abstract
Solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity. It is almost exclusively seen in females within the second and third decades of life with only small minority affecting children. Due to the paucity of the number of cases seen, the natural history of the disease is not fully understood. SPN tumors of the pancreas are usually found incidentally and usually have an excellent prognosis. We herein present a case of a 33-year-old lady diagnosed with SPN, who presented with abdominal fullness, two weeks post cesarean section.
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Affiliation(s)
- Steven Michael
- Surgery, Aga Khan University Medical College, Dar es Salaam, TZA
| | - Nadeem M Kassam
- Internal Medicine, Aga Khan University Medical College, Dar es Salaam, TZA
| | - Aidan Njau
- Surgery, Aga Khan University Medical College, Dar es Salaam, TZA
| | | | | | - Salim Surani
- Internal Medicine, Corpus Christi Medical Center, Corpus Christi, USA.,Internal Medicine, University of North Texas, Denton, USA
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Allam M, Hidalgo Salinas C, Machairas N, Kostakis ID, Watkins J, Fusai GK. Solid Pseudopapillary Neoplasms of the Pancreas: a Single-Center Experience and Review of the Literature. J Gastrointest Cancer 2021; 53:460-465. [PMID: 33877570 DOI: 10.1007/s12029-021-00638-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/11/2021] [Indexed: 12/15/2022]
Abstract
PURPOSE Pancreatic solid pseudopapillary neoplasms (SPNs) are rare borderline tumours mainly affecting young female patients. The number of patients diagnosed with SPNs has increased significantly in the last decades owing to the increased use of cross-sectional imaging investigating different abdominal symptoms, whilst a significant proportion are incidentally discovered during the process of evaluating other pathologies. We herein present our institutional experience of patients with SPN who underwent curative resection focusing on clinical, pathological features, and the long-term outcomes. METHODS All patients undergoing pancreatectomy in our institution for SPN from January 2010 until December 2018 were included. Clinical, perioperative, histological, and long-term outcomes were collected and analysed. RESULTS During the inclusion period, a total of 19 patients had a pathological diagnosis of SPNs after surgical resection. Sixteen of them were female (84%), while the median patient age was 30 (range 16-66) years. Nine patients (47%) underwent distal pancreatectomy and splenectomy, 2 (11%) underwent spleen preserving distal pancreatectomy, 6 (32%) underwent pancreatoduodenectomy, one (5%) underwent total pancreatectomy, and one (5%) central pancreatectomy. Seventeen patients underwent R0 resection. During a median follow-up of 23 months, no tumour recurrence or death was recorded. CONCLUSION In our experience, SPNs are rare tumours with low malignant potentials. Surgical resection remains the gold standard treatment and is associated with good prognosis.
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Affiliation(s)
- Moustafa Allam
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Camila Hidalgo Salinas
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Nikolaos Machairas
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK.
| | - Ioannis D Kostakis
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
| | - Jennifer Watkins
- Department of Cellular Pathology, Royal Free London NHS Foundation Trust, London, UK
| | - Giuseppe Kito Fusai
- Department of HPB Surgery and Liver Transplant, Royal Free Hospital NHS Foundation Trust, London, UK
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Ardengh JC, Lopes CV, Venco FE, Machado MA. Diagnosis of pancreatic solid pseudopapillary neoplasms using cell-blocks and immunohistochemical evaluation of endoscopic ultrasound-guided fine needle aspiration biopsy specimens. Cytopathology 2021; 32:50-56. [PMID: 32816310 DOI: 10.1111/cyt.12905] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2020] [Revised: 08/09/2020] [Accepted: 08/17/2020] [Indexed: 12/11/2022]
Abstract
INTRODUCTION Preoperative diagnostic imaging of pancreatic solid pseudopapillary neoplasms (SPNs) is challenging. A few studies have investigated the role of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) for the diagnosis of SPN. We investigated the diagnostic yield of cell-blocks and immunohistochemistry (IHC) for SPN using EUS-FNA specimens without cytological evaluation. PATIENTS AND METHODS We retrospectively analysed the histopathology records of patients with suspected SPN, who underwent EUS-FNA biopsy between January 1997 and January 2020. Diagnosis based on cell-blocks (haematoxylin-eosin staining with complementary IHC) was compared with the definitive surgical diagnosis. RESULTS This study included 25 patients (24 were women). Patients' mean age was 33.7 years (range 12-78 years). The most common symptom was abdominal pain. SPN was an incidental finding in 52% of the patients. The mean lesion size was 4.3 cm (range 1.2-11.4 cm), and the most common endosonographic features included solid-cystic (56%) or solid (40%) tumours. Final diagnoses included SPNs (n = 23) and non-functioning neuroendocrine tumours (n = 2). The overall accuracy of EUS-FNA was 80%. Tumour cells showed immunopositivity for β-catenin, CD10, CD99 and progesterone receptor (PR) in 93.7%, 87.5%, 83.3% and 66.6% of patients, respectively. No SPN showed immunopositivity for chromogranin A. CONCLUSIONS Intention-to-diagnose analysis showed that the diagnostic accuracy of EUS-FNA for SPNs using cell blocks and complementary IHC without cytological evaluation was fairly good. Evaluation of β-catenin, CD 10, CD99 and PR expression must be included in the IHC panel for diagnostic confirmation of SPNs using EUS-FNA biopsy specimens.
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Affiliation(s)
- José Celso Ardengh
- Division of Surgery and Anatomy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
- Endoscopy Unit, Hospital 9 de Julho, São Paulo, Brazil
| | - César Vivian Lopes
- Department of Gastroenterology and Digestive Endoscopy, Santa Casa Hospital, Porto Alegre, Brazil
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Kushner BS, Chatterjee D, Hammill C. Rare aggressive solid pseudopapillary neoplasm of the ovary with metastatic disease following surgical resection. BMJ Case Rep 2020; 13:13/12/e238136. [PMID: 33298496 DOI: 10.1136/bcr-2020-238136] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023] Open
Abstract
Extra-pancreatic solid pseudopapillary neoplasms (SPNs) are rare tumours with an overall favourable prognosis and low malignant potential. SPNs with metastatic spread, distant lymph node metastasis and extrapancreatic origin are exceedingly rare. Significant controversy regarding the treatment and the management of metastatic disease exists and, currently, there are no standardised guidelines or treatment recommendations for the use of adjuvant therapy. In this case report, the authors present a patient with widely metastatic SPN of likely ovarian origin with the invasion of the inguinal lymph nodes and multiple abdominal metastatic deposits. Using the currently available literature, the authors discuss treatment options for metastatic SPN of the ovary and highlight the need for continued research in this important field.
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Affiliation(s)
- Bradley S Kushner
- Department of Surgery, Washington University in Saint Louis School of Medicine, Saint Louis, Missouri, USA
| | - Deyali Chatterjee
- Department of Pathology, Washington University in Saint Louis School of Medicine, Saint Louis, Missouri, USA
| | - Chet Hammill
- Department of Surgery, Washington University in Saint Louis School of Medicine, Saint Louis, Missouri, USA
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Milanetto AC, Gais Zürcher AL, Macchi L, David A, Pasquali C. Pancreatic solid pseudopapillary neoplasm in male patients: systematic review with three new cases. Updates Surg 2020; 73:1285-1295. [PMID: 33123945 PMCID: PMC8397648 DOI: 10.1007/s13304-020-00905-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2020] [Accepted: 10/20/2020] [Indexed: 12/30/2022]
Abstract
Pancreatic solid pseudopapillary neoplasm (pSPN) is a rare exocrine neoplasm, which generally occurs in young women. This study analyses the clinical characteristics of pSPN in male patients through a systematic review of the literature, adding three new cases from our institution. We reviewed our experience in Pspns, and we performed a systematic review of pSPN of all articles published in English in PubMed and SCOPUS from 1980. Using the final included articles, we evaluated clinic-pathological features, surgical treatment and prognosis of male patients affected by pSPN. From the literature review and our cases, we collected 246 male patients with a proven pSPN. Mean age was 34.3 (range 4–78) years, with 26.2% patients younger than 18 years. Patients were asymptomatic in 35.9% of cases, despite a mean tumour size of 6.3 cm. In 63.7% of cases, the pSPN was located in the body–tail region. Distant metastases were reported at diagnosis in only 10 (4.1%) patients. A correct pre-operative diagnosis (including cytopathology) was provided in 53.6% of patients, with only 40 fine-needle aspiration/biopsy performed. Standard pancreatic resections represented 90.4% of surgical procedures. Beta-catenin and progesterone receptors were positive at immunostaining in 100% and 77.8% of cases, respectively. Fourteen (7.2%) patients relapsed after a mean disease-free survival of 43.1 months. After a mean follow-up of 47 (range 4–180) months, 89.5% of patients were alive and disease-free. Although rare, when dealing with a solid-cystic pancreatic mass, even in asymptomatic male patients, a pSPN should be considered as a possible diagnosis.
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Affiliation(s)
- Anna Caterina Milanetto
- Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 - 35128, Padua, Italy.
| | - Anna-Lea Gais Zürcher
- Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 - 35128, Padua, Italy
| | - Lorenzo Macchi
- Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 - 35128, Padua, Italy
| | - Alina David
- Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 - 35128, Padua, Italy
| | - Claudio Pasquali
- Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 - 35128, Padua, Italy
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A Morphologic and Immunohistochemical Comparison of Nuclear β-Catenin Expressing Testicular Sertoli Cell Tumors and Pancreatic Solid Pseudopapillary Neoplasms Supporting Their Continued Separate Classification. Am J Surg Pathol 2020; 44:1082-1091. [PMID: 32604170 DOI: 10.1097/pas.0000000000001527] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Some recent reports suggested that many Sertoli cell tumors, not otherwise specified (SCTs-NOS) of the testis were analogs of the solid pseudopapillary neoplasm (SPN) of the pancreas. One of the most relied on pieces of information for this assertion was the shared occurrence in both neoplasms of exon 3 mutations of the CTNNB1 gene, which was reflected by nuclear β-catenin expression. We, therefore, compared the morphologic and immunohistochemical features of 18 SCTs-NOS with strong, diffuse nuclear β-catenin expression with 16 SPNs that also showed such positivity. Although there were clear similarities in the light microscopic features of these neoplasms, there were also significant differences that included, in SCT-NOS and SPN, respectively: hollow tubules (53% vs. 0%), sheet-like growth (44% vs. 94%), circumscription (79% vs. 25%), corded or trabecular patterns (81% vs. 31%), formation of papillae or pseudopapillae (24% vs. 69%), growth in nests or clusters (94% vs. 50%), perivascular pseudorosettes (13% vs. 56%), and rhabdoid cytology (6% vs. 50%). Commonly shared morphologic features included signet-ring cells, pale or foamy cytoplasm, myxoid stroma, cyst formation, perivascular hyalinization, and globular or band-like basement membrane deposits. On immunohistochemical study, sex cord markers were frequently positive in SCTs-NOS (steroidogenic factor-1-94%; FOXL2-87%; SOX9-69%; calretinin-60%; Wilms tumor-1-38%; inhibin-29%) whereas all of these markers were negative in the SPNs. We conclude that even though SCT-NOS and SPN share some morphologic features and nuclear immunoreactivity for β-catenin, there remain differences, both morphologically and immunohistochemically, between these neoplasms to the degree that SCT-NOS should not be equated with pancreatic SPN.
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Chon HK, Choi KH, Kim TH. An Unusual Presentation of a Solid Pseudopapillary Tumor of the Pancreas Mimicking Adenocarcinoma. Clin Endosc 2020; 53:615-619. [PMID: 31752480 PMCID: PMC7548155 DOI: 10.5946/ce.2019.158] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Revised: 09/04/2019] [Accepted: 09/06/2019] [Indexed: 01/27/2023] Open
Abstract
Solid pseudopapillary tumors of the pancreas are rare and typically occur in young women. Compared with pancreatic adenocarcinoma, solid pseudopapillary tumors are characterized by notable indolent biological behavior associated with a favorable prognosis. Despite their large size, these tumors rarely metastasize. Even in cases of hepatic metastasis, most lesions are usually solitary in distribution and are amenable to resection. We report a case of a 55-year-old man with a small solid pseudopapillary tumor (≤3-cm diameter) mimicking a pancreatic adenocarcinoma, with multiple hepatic metastases. The diagnosis was confirmed by endoscopic ultrasound-guided fine-needle biopsy using a 22-G core needle. Unfortunately, rapid tumor progression led to patient mortality 5 months after diagnosis. To our knowledge, this is the first case report that describes a small solid pseudopapillary tumor of the pancreas with multiple hepatic metastasis and poor prognosis in a patient who was diagnosed with this condition at the time of initial diagnosis.
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Affiliation(s)
- Hyung Ku Chon
- Department of Internal Medicine, Wonkwang University College of Medicine and Hospital, Iksan, Korea
- Institute of Wonkwang Medical Science, Wonkwang University College of Medicine and Hospital, Iksan, Korea
| | - Keum Ha Choi
- Department of Pathology, Wonkwang University College of Medicine and Hospital, Iksan, Korea
| | - Tae Hyeon Kim
- Department of Internal Medicine, Wonkwang University College of Medicine and Hospital, Iksan, Korea
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Pant SR, Pokhrel NB, Chapagain P, Kansakar P. Different Methods of Resection of Solid Pseudopapillary Neoplasm of the Pancreas: A Case Series of Three Patients. Cureus 2020; 12:e7346. [PMID: 32328359 PMCID: PMC7170022 DOI: 10.7759/cureus.7346] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
Solid pseudopapillary neoplasm of the pancreas is one of the rarest forms of pancreatic neoplasm. It was also known as Franz's tumor or Hamoudi tumor until the World Health Organization (WHO) labeled it as a solid pseudopapillary tumor in 1996. It typically affects young non-Caucasian females in their second or third decade of life. Treatment involves complete excision of the tumor which results in a complete cure in most of the cases. Three cases of solid pseudopapillary neoplasm (diagnosis confirmed by cytology) in young females, each presenting with different symptoms were studied. Each of the three cases was found to have the neoplasm at different sites of the pancreas and was subjected to different resection procedures. The cases were followed up for at least a year and evaluated for recurrences/metastases. Solid pseudopapillary neoplasm remains one of the most misdiagnosed tumors. The diagnosis depends on radiology and cytology. With a very high five-year survival rate, surgical resection remains the treatment of choice. The type of surgical procedure depends on the site, size and local invasion of the tumor.
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Affiliation(s)
- Samriddha R Pant
- Surgery, Tribhuvan University Institute of Medicine, Kathmandu, NPL
| | - Nishan B Pokhrel
- Internal Medicine, Tribhuvan University Institute of Medicine, Kathmandu, NPL
| | | | - Prasan Kansakar
- Surgery, Tribhuvan University Institute of Medicine, Kathmandu, NPL
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Chagas VL, Rosman FC, Carvalho MDGDC. Solid pseudopapillary neoplasia of the pancreas: a review. ACTA ACUST UNITED AC 2020; 66:87-94. [PMID: 32130387 DOI: 10.1590/1806-9282.66.1.87] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2019] [Accepted: 09/01/2019] [Indexed: 01/20/2023]
Abstract
OBJECTIVES To review the literature and the diagnosis of conventional histopathological routine and immunohistochemistry of the cases diagnosed with Solid Pseudopapillary Neoplasm of the Pancreas (SPNP). METHODS The review of the literature was done using the Pubmed and solid Google-Scholar databases, through the historical, clinical aspects and diagnostic methods of SPNP. The review of SPNP cases diagnosed in the University Hospital Clementino Fraga Filho was carried out from 1977 to 2018. RESULTS Intratumoral phenotypic heterogeneity of SPNP was evidenced in the cases studied, taking into account macroscopic, microscopic, and immunohistochemical patterns. CONCLUSIONS The results show the importance of the examination of several fragments obtained from different regions of the neoplasia since not all of them present the same molecular alterations.
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Affiliation(s)
- Vera Lucia Chagas
- . Professora no Departamento de Patologias, Faculdade de Medicina do Hospital Clementino Fraga Filho da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brasil
| | - Fernando Colonna Rosman
- . Professor do Departamento de Patologias, Faculdade de Medicina do Hospital Clementino Fraga Filho da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brasil
| | - Maria da Gloria da Costa Carvalho
- . Pesquisadora no Departamento de Patologias, Faculdade de Medicina do Hospital Clementino Fraga Filho da Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brasil
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Farhat W, Ammar H, Amine Said M, Mizouni A, Bouazzi A, Abdessaied N, Ben Mabrouk M, Ben Ali A. Solid pseudopapillary neoplasm of the pancreas: a report of 10 cases and literature review. ANZ J Surg 2020; 90:1683-1688. [PMID: 31989788 DOI: 10.1111/ans.15701] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2019] [Accepted: 01/07/2020] [Indexed: 12/13/2022]
Abstract
BACKGROUND Solid pseudopapillary tumour of the pancreas (SPTP) is a rare pancreatic tumour characterized by a non-specific clinical presentations and vague radiologic features. The aim of this study is to identify these tumours from other pancreatic neoplasms because complete resection is curative in most cases and provides long-term survival. METHODS A retrospective analysis of patients operated for SPTP between January 2000 and December 2018 was conducted. The collected data included age, gender, clinical findings, laboratory tests, radiological findings, anatomopathological examination, immunohistochemistry results, surgical treatment, mortality, morbidity and recurrence. RESULTS Ten cases of SPTP have been diagnosed between January 2000 and December 2018 representing 5.1% of all pancreatic tumours operated during this period (male/female: 2/8; median age 41.2 years; range 19-78 years). The most common symptom was abdominal pain and physical examination was normal in four of 10 cases. The most common tumour localization was the tail of the pancreas. The main tumour size was 7.2 cm (range 2-15 cm). One patient had abdominal disseminated disease. Surgical interventions were distal pancreatectomy in five cases, enucleation in one case, cephalic duodeno-pancreatectomy in two cases, central pancreatectomy in one case and pancreatic biopsy in one case. Only one patient received adjuvant chemotherapy. During follow-up, one patient died after 12 months and another developed unique hepatic metastasis that was resected. CONCLUSION Although it is delayed in diagnosis, the overall prognosis of these tumours remains good even with local recurrence and metastasis. Complete surgical resection is the treatment of choice even in cases of recurrence.
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Affiliation(s)
- Waad Farhat
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Houssem Ammar
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Mohamed Amine Said
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Abdelkader Mizouni
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Amal Bouazzi
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Nihed Abdessaied
- Department of Pathology, Farhat Hached Hospital, University of Sousse, Sousse, Tunisia
| | | | - Ali Ben Ali
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
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Guo T, Wang L, Xie P, Zhang Z, Yu Y. Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience. Cancer Manag Res 2020; 12:581-588. [PMID: 32158262 PMCID: PMC6986403 DOI: 10.2147/cmar.s238527] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2019] [Accepted: 01/14/2020] [Indexed: 12/17/2022] Open
Abstract
Purpose To investigate the diagnosis, surgical treatment, and pathology of solid pseudopapillary tumors of the pancreas in our institution. Patients and Methods We retrospectively analyzed the demographic details, clinical features, imaging findings, and pathological findings of 87 patients with a confirmed diagnosis of solid pseudopapillary tumors of the pancreas (SPTP) and underwent surgery in Tongji Hospital of Tongji Medical College, over a period of 8 years from 2011 to 2018. Results Our study involved a total of 87 patients (16 males and 71 females) with a mean age of 31.3±13.1 years (range: 10–61 years). The main compliant was abdominal pain or discomfort (n=49) and the median tumor size was 58.6±31.7 mm (range: 16–156mm). Tumors were located in the head (27 patients, 31%), the neck (13 patients, 15%), and the body and tail (47 patients, 54%). There were no significant differences between the patients in terms of sex, age, or tumor location. Partial pancreatectomy was performed in 79 patients, enucleation in six patients, and total pancreatectomy in two patients. R0 resection was achieved in 86 patients. The postoperative morbidity was 36.8%, and the main complication was pancreatic fistula. Pathological examination and immunohistochemical markers were used to provide a final diagnosis. The main follow-up period was 46 months (range: 13–97 months). At the end of the follow-up period, 86 patients were alive and had not experienced recurrence; one patient was lost to follow-up. Conclusion The accurate diagnosis of SPTP is vital. Our data showed that surgical resection is safe and associated with low morbidity and mortality rates. Pathological findings can play an important role in diagnosis and long-term survival.
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Affiliation(s)
- Tong Guo
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Lu Wang
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Peng Xie
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Zhiwei Zhang
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Yahong Yu
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
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