Forty-Five Pancreatic Gastrinomas in Multiple Endocrine Neoplasia Type 1: A Case Report

Abstract

The patient was a 49-year-old woman who had been diagnosed with multiple endocrine neoplasia type 1 (MEN-1). Dynamic contrast-enhanced CT indicated several hypervascular tumors in the pancreas. Endoscopic US showed low-echoic lesions in the uncinate process and the tail and body of the pancreas, and a diagnosis of pancreatic neuroendocrine tumor was made by fine-needle aspiration. A selective arterial secretagogue injection test indicated an increased serum gastrin level and was considered positive in all feeding arteries of the pancreas. Under a diagnosis of MEN-1 with multiple pancreatic gastrinomas, total pancreatectomy with D2 lymph node dissection was performed. Histopathological findings showed 45 or more microgastrinomas in the whole pancreas with lymph node metastases. At 21-month follow-up, the patient remained disease-free. This case shows the need for selection of appropriate surgical treatment given that gastrinomas in MEN-1 have higher malignancy and are characterized by multiple microlesions, rather than sporadic gastrinomas.