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

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

Int J Surg Case Rep

Department of Surgery, Division of Surgical Oncology, University of California San Francisco, San Francisco, CA, USA. Electronic address:

Published: June 2021

AI Article Synopsis

Article 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 pancreatosplenectomy (RAMP) and recovered well post-operatively with complete resolution of his diarrhea. Immunohistochemistry showed multiple pure PPomas.

Clinical Discussion: This case highlights the unique propensity for multifocal disease in patients with MEN1 mutations and the utility of functional imaging by somatostatin analogs, i.e., Ga68-DOTATATE PET/CT, in order to perform oncologic laparoscopic pancreatic resections.

Conclusion: PPomas in the setting of MEN1 mutations are a unique clinical entity due to their diverse associated clinical syndromes and propensity for multifocal disease.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8193151PMC
http://dx.doi.org/10.1016/j.ijscr.2021.106008DOI Listing

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