[Pheochromocytoma, first manifestation of Von Hippel-Lindau disease: a possibility to be considered].

Von Mippel-Lindau (VML) disorder is an autosomal dominant disease characterized by the almost constant development of hemangioblastomas in the central nervous system (cerebellum, spinal cord and retina). In addition, various types of tumors including renal cell carcinomas, pancreatic cysts and pheochromocytomas are frequently observed in VML gene carriers. Linkage of the VML locus to the RAF-1 oncogene on the short arm of chromosome 3 (3p25-26) has been recently reported