Ninety-seven gene carriers of von Hippel-Lindau syndrome (HLS) were studied at the University of Freiburg, Germany, to evaluate the prevalence and location of CNS lesions. Hemangioblastomas of the CNS were found in 43 patients (44%). Of a total of 93 hemangioblastomas found in 43 patients (44%), 74% were intracranial and 26% were in the spinal cord. Seventy-five percent were cystic and 25% solid. Multiple lesions occurred in 42% of HLS hemangioblastomas but in none of 51 CNS hemangioblastomas without HLS. Cerebella hemangioblastomas were the major cause of death in 82% of the patients with HLS. 
COMMENT. Other manifestations of von Hippel-Lindau syndrome were as follows: retinal angiomatosis - 44%, renal cysts - 47%, pheochromocytoma - 5%, pancreatic cysts - 30%, and epididymal cystadenoma - 15%. The MRI and gadolinium-enhanced MRI have improved the detection of small CNS lesions which are the initial manifestations of the syndrome and the largest percentage of patients. Multifocal tumor development and recurrence are serious problems in management and prognosis.