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Ubiquity Press, Journal of the Belgian Society of Radiology, 3(94), p. 140, 2011

DOI: 10.5334/jbr-btr.551

BMJ Publishing Group, Journal of Neurology, Neurosurgery and Psychiatry, 10(55), p. 898-901, 1992

DOI: 10.1136/jnnp.55.10.898

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Central nervous system lesions in von Hippel-Lindau syndrome.

Journal article published in 1992 by H. P. H. Neumann, A. K. Wakhloo, B. Volk, H. R. Eggert, S. Traen, R. Scheremet, M. Schumacher, P. Seynaeve, R. Seynaeve, M. Mohadjer, Wakhloo Ak, L. Cardoen ORCID, K. Carels, U. Hettmannsperger, P. Riegler and other authors.
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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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.