Multifocal pancreatic PPoma in the setting of MEN1: Case report and review of literature. (June 2021)

• Record Type: Journal Article
• Authors: Mosenia, Arman; Ward, Casey; Yee, Alisa; Qorbani, Amir; Corvera, Carlos
• Is Part Of: International journal of surgery case reports. Volume 83(2021)
• Publication Date: 2021-06
• Subjects: CgA chromogranin A -- ISGPS International Study Group in Pancreatic Surgery -- MEN1 multiple endocrine neoplasia -- pNET pancreatic neuroendocrine tumors -- PPoma pancreatic polypeptide-producing tumor -- RAMP radical antegrade modular pancreatosplenectomy -- VIPoma vasoactive intestinal polypeptide-secreting tumors; Case report -- PPoma -- MEN1 -- Multifocal -- pNET -- Ga68-DOTATATE PET/CT; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2021.106008
• Languages: English
• ISSNs: 2210-2612

Abstract

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. Highlights: PPomas have no pathognomonic clinical syndrome associated with them. Patients with MEN1 have a propensity for multifocal pNETs. DOTATE PET/CT is vital adjunct to pancreatic protocol CT for operative planning. Intra-operative ultrasound makes laparoscopic multi-focal pNET resection feasible. DOTATATE PET/CT surveillance is necessary due to high risk for recurrence in MEN1.