A case of multiple pancreatic neuroendocrine tumors with von Hippel-Lindau disease

Abstract

A 19-year-old woman, who presented with excessive sweating and hypertension, was diagnosed with multiple pancreatic neuroendocrine tumors (PNET) and adrenal pheochromocytomas. Based on tumors in multiple organs and her family history, we diagnosed von Hippel-Lindau disease. Distal pancreatectomy (body-tail), enucleation of the tumor in the pancreatic head, and adrenalectomy were performed, avoiding total pancreatectomy in terms of preservation of pancreatic function. Histopathological examination revealed multiple PNETs in addition to those recognized before the surgery, but they were thought to be too small to detect. VHL patients often have multiple tumors including PNET synchronously and metachronously, therefore careful consideration of the operative course and follow-up is required.