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Glioblastoma multiforme in four siblings: A cytogenetic and molecular genetic study

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Summary

The familial occurrence of gliomas, in the absence of well-defined neurological tumor syndromes such as the neurofibromatoses, is uncommon. We present a family of ten children in which the four eldest suffered from gliomas. Three of these siblings had histologically verified glioblastoma multiforme, and one patient also had an intestinal non-Hodgkin's lymphoma, but there were no stigmata or family history of a neurological tumor syndrome. Cytogenetic studies of the proband revealed a normal karyotype. Molecular genetic analysis of the proband's glioblastoma revealed two mutations in the p53 tumor suppressor gene, but these were not present in the germline DNA, mutations were not detected in the MTS1 gene in the tumors or in the germline DNA. These findings suggest that a genetic factor may be responsible for the clustering of glial tumors in this family, but it is unlikely that the genetic alteration is mutation of the p53 gene. The data are discussed in light of the literature on familial brain tumors.

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Authors and Affiliations

  1. Department of Neurosurgery, University Hospital Groningen, Oostersingel 59, P.O. Box 30001, 9700 RB, Groningen, The Netherlands

    Clemens M. F. Dirven & K. Gwan Go

  2. Department of Pathology, University Hospital Groningen, Oostersingel 59, P.O. Box 30001, 9700 RB, Groningen, The Netherlands

    Willemina M. Molenaar

  3. Department of Human Genetics, University Hospital Nijmegen, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands

    Joep Tuerlings

  4. Molecular Neuro-Oncology Laboratory, Department of Pathology (Neuropathology) and Neurosurgical Service, Massachusetts General Hospital, USA

    David N. Louis

  5. Harvard Medical School, Boston, MA, USA

    David N. Louis

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  1. Clemens M. F. Dirven
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  2. Joep Tuerlings
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  4. K. Gwan Go
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  5. David N. Louis
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Dirven, C.M.F., Tuerlings, J., Molenaar, W.M. et al. Glioblastoma multiforme in four siblings: A cytogenetic and molecular genetic study. J Neuro-Oncol 24, 251–258 (1995). https://doi.org/10.1007/BF01052841

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