Pancreatic neuroendocrine tumor and renal cell carcinoma in a patient: Von Hippel-Lindau Disease

Abstract

Von Hippel-Lindau disease is an autosomal dominant multisystemic cancer syndrome due to a mutation of the Von Hippel-Lindau disease tumor suppressor gene on chromosome 3. The major lesions in Von Hippel-Lindau disease disease include hemangioblastomas in the central nervous system and retina, clear cell renal cell carcinomas, pheochromocytomas, pancreatic tumors, epididymal cystadenomas, endolymphatic sac tumors, carcinoid tumors, and multiple cysts of the kidney, pancreas and epididymis. Most patients have pancreatic involvement in Von Hippel-Lindau disease disease. Pancreatic disease of Von Hippel-Lindau disease includes benign cysts, microcystic adenomas, pancreatic neuroendocrine tumors , and pancreatic metastases of renal cell carcinoma. Pancreatic neuroendocrine tumors should be differentiated from other hypervascular tumors and especially with clear cell morphology, such as renal cell carcinoma and microcystic adenomas. Herein, we report a patient with the diagnosis of Von Hippel-Lindau disease who had malignant neuroendocrine tumorof the pancreas and renal adenocarcinoma.