Revista ABCd (São Paulo). 08 jul, 2025

PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS

José Donizeti MEIRA-JÚNIOR
Gustavo Gonçalves YOGOLARE
Daniel de Paiva MAGALHÃES
Guilherme Naccache NAMUR
Fabio Guilherme CAMPOS
Vanderlei SEGATELLI
Sergio Carlos NAHAS
Jose JUKEMURA
DOI: https://doi.org/10.1590/0102-672020220002e1718

BACKGROUND:

Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well.

AIM:

The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare.

METHODS:

We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female).

RESULTS:

ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas.

CONCLUSION:

Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.


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ABCD – BRAZILIAN ARCHIVES OF DIGESTIVE SURGERY is a periodic with a single annual volume in continuous publication, official organ of the Brazilian College of Digestive Surgery - CBCD. Technical manager: Dr. Francisco Tustumi | CRM: 157311 | RQE: 77151 - Cirurgia do Aparelho Digestivo

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