[Hippel-Lindau syndrome: morphology and immunohistochemistry of tumors related to this syndrome].

Hippel Lindau syndrome (HLS), inherited as a simple dominant trait, is characterized by angiomatosis in the brain and retina, and also by cysts and tumours in various abdominal organs. Microscopically there is a striking morphological similarity between some of these tumours and especially between those in the brain (hemangioblastomas) and in the kidneys (renal cell carcinomas). Biopsy and autopsy material from two patients with HLS was examined chiefly by immunohistochemical methods, to investigate further the origin of these tumours. The cerebral hemangioblastomas of both patients showed tumour cells with a positive immunohistochemical reaction for neuron-specific enolase (NSE), suggesting a neural or neuroendocrine origin, while corresponding investigation of the kidney tumours did not produce similar clear results. Systemic immunohistochemical investigation of all tumours related to this syndrome is recommended.