Systematic Reviews
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Jul 15, 2025; 17(7): 106701
Published online Jul 15, 2025. doi: 10.4251/wjgo.v17.i7.106701
Adult pancreatoblastoma: Systematic review of the literature and case report of a young adult patient
Jacob T Harris, Steve Gurley, Erkut Borazanci
Jacob T Harris, Internal Medicine, HonorHealth Thompson Peak Medical Center, Scottsdale, AZ 85255, United States
Steve Gurley, Department of Pathology, HonorHealth Osborn Medical Center, Scottsdale, AZ 85251, United States
Erkut Borazanci, Department of Oncology, HonorHealth Research Institute, Scottsdale, AZ 85258, United States
Author contributions: Harris JT performed the research and wrote the manuscript; Harris JT and Borazanci E designed the research; Harris JT, Gurley S, and Borazanci E analyzed the data and edited the manuscript; and all authors thoroughly reviewed and endorsed the final manuscript.
Supported by the Honor Health Foundation, Michael and Mary Ellen Francis; and the Seena Magowitz Foundation.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
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: Jacob T Harris, MD, Internal Medicine, HonorHealth Thompson Peak Medical Center, 7400 E Thompson Peak Parkway, Scottsdale, AZ 85255, United States. jacharris@honorhealth.com
Received: March 5, 2025
Revised: April 30, 2025
Accepted: June 16, 2025
Published online: July 15, 2025
Processing time: 131 Days and 12.1 Hours
Abstract
BACKGROUND

Adolescent/adult pancreatoblastoma (PB) is an uncommon malignant pancreatic tumor. The paucity of data stemming from the rarity of this disease leads to minimal generalized guidelines regarding its diagnosis and treatment. There is a limited number of case reports in the literature and there has been no recent analysis of the literature to consolidate their common features. The purpose of the featured study is to review the available cases of adolescent/adult PB and analyze the common genetic features, histologic features, treatment regimens, tumor sizes, tumor locations, and areas of metastasis to advance ongoing research and better understand and treat this rare condition.

AIM

To present a patient case and systematically review all available cases in the literature to consolidate the common physical, genetic, and histologic features of PB.

METHODS

This is a systematic review of the literature with a case study. A total of 89 patient cases were discovered in the literature database for adolescent/adult PB, all of which were reviewed and are included in our research. Patients aged 16-18 were considered adolescent and patients aged greater than 18 were considered adult. Adolescents and adults were grouped together for the purpose of this study. The patient from the case report was seen in a community hospital setting.

RESULTS

The 89 cases analyzed from the literature were found in 51 references (our case report included), which were consolidated into the six categories mentioned above. A plurality of references reports PB in the head of the pancreas, 4.0-10.0 cm in size, and with the most common site of metastasis to the liver. Histology studies most commonly included acinar groups, squamous corpuscles/nests, cytokeratin, chromogranin, trypsin, chymotrypsin, and synaptophysin. Genetic studies most commonly included adenomatous polyposis coli, B-cell lymphoma/leukemia 10, catenin beta 1, and Wnt/beta-catenin mutations. The mainstay of treatment was surgery with chemotherapy typically including cisplatin, carboplatin, doxorubicin, 5 fluorouracil, mitomycin, bleomycin, gemcitabine, and vindesine. Radiation was also often used.

CONCLUSION

Common pancreatoblastoma features include acinar groups, chromogranin, chymotrypsin, squamous corpuscles, synaptophysin and trypsin on histology and adenomatous polyposis coli, B-cell lymphoma/leukemia 10, catenin beta 1, and Wnt/beta-catenin genetic mutations.

Keywords: Adult pancreatoblastoma; Pancreatoblastoma genetic features; Pancreatoblastoma histological features; Pancreatoblastoma treatment; Pancreatoblastoma physical features; Adult pancreatoblastoma case report; Adult pancreatoblastoma systematic review

Core Tip: Pancreatoblastoma is exceedingly rare in the adolescent/adult population. This is a systematic review of all the 89 available cases in the literature as well as a case presentation. Notable results of this study include: (1) Common histologic features of acinar groups, squamous corpuscles/nests, cytokeratin, chromogranin, trypsin, chymotrypsin, and synaptophysin; (2) Common genetic features of adenomatous polyposis coli, B-cell lymphoma/leukemia 10, catenin beta 1, and Wnt/beta-catenin mutations; and (3) Chemotherapeutic regimens including cisplatin, carboplatin, doxorubicin, 5 fluorouracil, mitomycin, bleomycin, gemcitabine, and vindesine. Future therapies should focus on targeting the Wnt pathway.