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Multifocal pancreatic PPoma in the setting of MEN1: Case report and review of literature.


ABSTRACT:

Introduction and importance

Functioning pancreatic neuroendocrine tumors (pNETs) that express pancreatic polypeptide-PPomas-do not yet have a pathognomonic clinical syndrome associated with them due to their overall rarity and diverse symptoms. Moreover, in patients with MEN1, the often multifocal nature of pNETs presents a unique clinical issue.

Case presentation

We report a case of a 22-year-old man with a known MEN1 gene mutation who was suffering from severe diarrhea (7-8 bowel movements per day) and was found to have only elevated PP levels on biochemical work-up. Ga68-DOTATATE PET/CT showed multifocal tumors in the body and tail of the pancreas that were not evident on contrast-enhanced CT. The patient underwent a successful laparoscopic radical antegrade modular pancr

SUBMITTER: Mosenia A 

PROVIDER: S-EPMC8193151 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature