Von Hippel Lindau syndrome

Abstract

Von Hippel Lindau Syndrome(VHL) is an autosomal dominant neoplastic syndrome, characterized by hemangioblastomas of the central nervous system, retinal angiomas, papillary renal cell carcinomas, visceral cysts, pheochromocytoma, and islet cell tumors. This case report present a patient who has many of this manifestations: cerebellum hemangioblastoma, pheochromocytoma, islet cell tumor with carcinoid syndrome, biliar cysts. Clinical management for this patient with VHL disease is complicated, and the most important seems to be the amelioration of clinical symptoms, improvement of life quality, and prolongation of overall survival.