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Central nervous system lesions in von Hippel-Lindau syndrome.
  1. H P Neumann,
  2. H R Eggert,
  3. R Scheremet,
  4. M Schumacher,
  5. M Mohadjer,
  6. A K Wakhloo,
  7. B Volk,
  8. U Hettmannsperger,
  9. P Riegler,
  10. P Schollmeyer
  1. Department of Medicine, Albert-Ludwigs-University, Freiburg, Germany.

    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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