Fibrous meningioma in a patient with von Hippel-Lindau disease

A genetic analysis. Case report

L. S. Governale, Alexander Vortmeyer, Z. Zhuang, E. H. Oldfield

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Meningioma has been included in the constellation of tumors associated with von Hippel-Lindau (VHL) disease in previously published reports. It is unclear whether these tumors are an uncommon component of VHL disease or are more readily detected in these patients because of the frequency with which they undergo central nervous system imaging as part of the routine management of VHL disease. The authors report the case of a patient with VHL disease in whom a progressively enlarging supratentorial mass developed and was diagnosed as a hemangioblastoma because of its appearance on serial magnetic resonance images. At surgery the tumor displayed the typical features of a meningioma and was given the histological diagnosis of fibrous meningioma. Single-stranded conformational polymorphism analysis of the tumor DNA revealed a loss of heterozygosity at the neurofibromatosis Type 2 gene locus, known to be associated with sporadically occurring meningiomas. Despite this finding, the VHL gene locus on the allele from the patient's unaffected parent was normal. Thus it is unlikely that the occurrence of this patient's fibrous meningioma was associated with underlying VHL disease. Given the high frequency of neuroimaging sessions in patients with VHL disease, some supratentorial lesions that have been given radiological diagnoses of hemangioblastomas may be incidental meningiomas.

Original languageEnglish (US)
Pages (from-to)1045-1049
Number of pages5
JournalJournal of neurosurgery
Volume95
Issue number6
DOIs
StatePublished - Jan 1 2001
Externally publishedYes

Fingerprint

von Hippel-Lindau Disease
Meningioma
Hemangioblastoma
Neoplasms
Neurofibromatosis 2 Genes
Neurofibromatosis 2
Single-Stranded Conformational Polymorphism
Loss of Heterozygosity
Neuroimaging
Magnetic Resonance Spectroscopy
Central Nervous System
Alleles
DNA
Genes

Keywords

  • Loss of heterozygosity
  • Meningioma
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Fibrous meningioma in a patient with von Hippel-Lindau disease : A genetic analysis. Case report. / Governale, L. S.; Vortmeyer, Alexander; Zhuang, Z.; Oldfield, E. H.

In: Journal of neurosurgery, Vol. 95, No. 6, 01.01.2001, p. 1045-1049.

Research output: Contribution to journalArticle

Governale, L. S. ; Vortmeyer, Alexander ; Zhuang, Z. ; Oldfield, E. H. / Fibrous meningioma in a patient with von Hippel-Lindau disease : A genetic analysis. Case report. In: Journal of neurosurgery. 2001 ; Vol. 95, No. 6. pp. 1045-1049.
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