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World J Hepatol. Aug 27, 2021; 13(8): 896-903
Published online Aug 27, 2021. doi: 10.4254/wjh.v13.i8.896
Solid pseudopapillary neoplasm of the pancreas
Ayo O Omiyale
Ayo O Omiyale, Department of Cellular Pathology, Imperial College Healthcare NHS Trust, Charing Cross Hospital, London W6 8RF, United Kingdom
Author contributions: Omiyale AO reviewed the literature and wrote the manuscript.
Conflict-of-interest statement: The author declares no conflict of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ayo O Omiyale, MBChB, MPH, Doctor, Department of Cellular Pathology, Imperial College Healthcare NHS Trust, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, United Kingdom. ayodeji.omiyale@nhs.net
Received: March 2, 2021
Peer-review started: March 2, 2021
First decision: May 2, 2021
Revised: May 16, 2021
Accepted: August 3, 2021
Article in press: August 3, 2021
Published online: August 27, 2021
Processing time: 170 Days and 16.9 Hours
Abstract

Solid pseudopapillary neoplasms are rare. This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas, including the epidemiology, cytology, molecular pathology, differential diagnosis, treatment, and prognosis. Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns. Solid pseudopapillary neoplasms occur predominantly in young women. Although solid pseudopapillary neoplasms can occur throughout the pancreas, they arise slightly more frequently in the tail of the pancreas. The aetiology is unknown. Extremely rare cases have been reported in the setting of familial adenomatous polyposis. There are no symptoms unique to solid pseudopapillary neoplasms, however, the most common symptom is abdominal pain or discomfort. The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour. Typically, well-demarcated masses with variably solid and cystic appearances. Microscopically, these tumours are composed of epithelial cells forming solid and pseudopapillary structures, frequently undergoing haemorrhagic cystic degeneration. Typically, these tumours express nuclear and/or cytoplasmic β-catenin. Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1, the gene encoding β-catenin. The overall prognosis is excellent, and most patients are cured by complete surgical resection.

Keywords: Cancer of pancreas; Pancreatic neoplasms; Solid pseudopapillary neoplasm of the pancreas; Non-ductal pancreatic tumours; Pancreas

Core Tip: Solid pseudopapillary neoplasms are low-grade malignant tumours that mimic other solid cellular neoplasms of the pancreas. This article summarizes the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas including the epidemiology, molecular pathology, differential diagnosis, treatment, and prognosis.