Review
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Mar 15, 2024; 16(3): 614-629
Published online Mar 15, 2024. doi: 10.4251/wjgo.v16.i3.614
Unraveling the enigma: A comprehensive review of solid pseudopapillary tumor of the pancreas
Ye-Cheng Xu, De-Liang Fu, Feng Yang
Ye-Cheng Xu, De-Liang Fu, Feng Yang, Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
Author contributions: Xu YC and Yang F contributed to data acquisition, and drafting the article; Fu DL and Yang F contributed to the conception and design of this paper; all authors contributed to the final approval of the version to be published.
Conflict-of-interest statement: We declare no conflicts of interest.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Feng Yang, MD, PhD, Doctor, Surgeon, Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, No. 12 Central Urumqi Road, Shanghai 200040, China. yffudan98@126.com
Received: November 9, 2023
Peer-review started: November 9, 2023
First decision: December 18, 2023
Revised: December 28, 2023
Accepted: January 16, 2024
Article in press: January 16, 2024
Published online: March 15, 2024
Processing time: 124 Days and 7.2 Hours
Abstract

Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly observed in young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and subsequent Wnt-signaling pathway activation are the leading molecular features. Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies. Surgical resection remains the mainstay treatment. Risk models, such as the Fudan Prognostic Index, show promise as predictive tools for assessing the prognosis of SPTP. Establishing three types of metachronous liver metastasis can be beneficial in tailoring individualized treatment and follow-up strategies. Despite advancements, challenges persist in understanding its etiology, establishing standardized treatments for unresectable or metastatic diseases, and developing a widely recognized grading system. This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology, clinical presentation, pathology, molecular characteristics, diagnostic methods, treatment options, and prognostic factors.

Keywords: Pancreas; Solid pseudopapillary tumor; β-catenin; Endoscopic ultrasound; Surgery; Recurrence; Liver metastasis; Prognostic prediction

Core Tip: Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly affecting young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and Wnt signaling pathway activation are key molecular features. Accurate preoperative diagnosis relies on imaging and endoscopic biopsies. Surgical resection is the main treatment, and prognostic models like Fudan Prognostic Index aid in prognosis assessment. Challenges in understanding its etiology, establishing treatments for unresectable/metastatic disease, and developing a standardized grading system persist. This comprehensive review aims to consolidate current knowledge on epidemiology, clinical presentation, pathology, molecular features, and treatment options for SPTP.