Retinocerebellar angiomatosis, phakomatosis, autosomal dominant (variable penetrance), haemangioblastoma: most frequent cause of death, cerebellar (most common), also medullary and spinal, retinal angiomatosis (45%), renal cell carcinoma: 2nd most common cause of death, pheochromocytoma (17%), cortical renal cysts (75%), cysts in virtually any organ, renal/liver haemangioma/adenoma, pancreatic cystic neoplasms, isleT-cell tumours, paraganglioma
(12 Dec 1998)