Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas

Abstract

Abstract Background Gastrinomas are functional endocrine duodenopancreatic tumours and are responsible for Zollinger–Ellison syndrome (ZES). Clinical presentation, localization techniques and operative management were reviewed. Methods An electronic search of the Medline database was undertaken for articles published in English between January 1987 and May 2007. This timeframe was chosen because of the fundamental changes in operative strategy, antisecretory therapy and localization techniques during this period. Results and conclusion Most gastrinomas are located in the ‘gastrinoma triangle’, comprising the head of the pancreas, and the first and second parts of the duodenum. Some 20 per cent of gastrinomas occur in association with multiple endocrine neoplasia type 1 (MEN1) and 50–60 per cent of tumours are malignant at the time of diagnosis. Biochemical evidence justifies operation of which duodenotomy is an essential part. Only complete tumour resection allows 5- and 10-year survival rates of 90 per cent. Pylorus-preserving pancreaticoduodenectomy may be the procedure of choice for MEN1–ZES.