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. 1992 Oct;55(10):898-901.
doi: 10.1136/jnnp.55.10.898.

Central nervous system lesions in von Hippel-Lindau syndrome

H P Neumann  1 , H R Eggert, R Scheremet, M Schumacher, M Mohadjer, A K Wakhloo, B Volk, U Hettmannsperger, P Riegler, P Schollmeyer, et al.
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Central nervous system lesions in von Hippel-Lindau syndrome

H P Neumann et al. J Neurol Neurosurg Psychiatry. 1992 Oct.

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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