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Central Nervous System Lesions in von Hippel-Lindau Syndrome JNNP 55:898-901, Neumann,H.P.H.,et al, 1992 See this aricle in Pubmed Article Abstract CNS manifestations were studied in 97 gene carriers of von Hippel-Lindau syndrome(HLS).Haemangioblastomas of the CNS were found in 43 patients (44%),23 females and 20 males.The mean age at diagnosis was 39 years(12-73 years).A total of 93 haemangioblastomas were detected of which 74%were intracranial and 26%were located in the spinal cord;75%were predominantly cystic and 25%presented as solid lesions.Multiple lesions were found in 42%of HLS-associated haemangioblastomas,but in none of 51 patients with CNS haemangioblastoma without HLS.Haemangioblastoma was the cause of death in 82%of patients with HLS.Although microsurgery has considerably improved post-operative results,multifocal tumour development and recurrence remain a serious problem in the clinical management of HLS gene carriers.

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