Pancreatic Lesions in von Hippel-Lindau Syndrome: Report of a Case

Abstract

Von Hippel-Lindau (VHL) syndrome is a rare autosomal inherited disorder. A germline mutation predisposes carriers to the development of multiple tumors, the most common of which are hemangioblastoma, renal cell carcinoma, pheochromocytoma, and islet cell and cystic tumors of the pancreas, the kidney, and epididymis. Visceral manifestations, such as cystadenoma of the pancreas, occur late in its course. We report the case of a 20-year old woman who presented with vomiting and dizziness, most probably caused by a palpable mass in the upper abdomen, diagnosed as pancreatic cysts. She had undergone surgery for cerebellar hemangioblastoma 4 years previously. In view of her young age, preservation of pancreatic function was crucial because of the expense of enzyme therapy and the difficulties with follow-up. Therefore, we performed cyst enucleation and distal pancreatectomy, which relieved her symptoms. She has been well with no sign of recurrence for 18 months. This case highlights that a well-prepared surgical procedure can cure a patient with pancreatic cysts caused by VHL, living in difficult socioeconomic conditions.