Familial glioblastoma clustering in adult patients: a case report of two non-twin siblings and review of the literature
Authors Sander C, Reuschel V, Eisenlöffel C, Nestler U, Meixensberger J
Received 14 January 2019
Accepted for publication 3 April 2019
Published 5 July 2019 Volume 2019:12 Pages 205—211
Checked for plagiarism Yes
Review by Single-blind
Peer reviewers approved by Dr Amy Norman
Peer reviewer comments 2
Editor who approved publication: Professor Ronald Prineas
Caroline Sander,1 Vera Reuschel,2 Christian Eisenlöffel,3 Ulf Nestler,1 Jürgen Meixensberger1
1Department of Neurosurgery, University Hospital Leipzig, Leipzig 04103, Germany; 2Department of Neuroradiology, University Hospital Leipzig, Leipzig 04103, Germany; 3Institute of Neuropathology, University Hospital Leipzig, Leipzig 04103, Germany
Purpose: Familial glioblastoma multiforme (gbm) has been described in children with hereditary tumor syndromes. The occurrence of gbm in adult members of the same family and in the absence of tumor syndromes is extremely rare. We describe the cases of a brother and a sister with multifocal gbm diagnosed at the age of 63 years. We discuss three further paired gbm in adult patients from the literature.
Patients and results: The sister was diagnosed with multifocal primary gbm in 2014 at the age of 63 years and 6 months. In 2018, her younger brother had to be operated on for a multifocal primary gbm at the age of 63 years and 9 months. Extended neuropathological examination revealed most markers to be similar, except for the percentage of O6,-methylguanine-DNA methyltransferase promotor methylation, the presence of intratumoral immune cells and the immunohistochemical expression of C12ORF75. Comparison with further published cases of familial adult GBM reveals that most of these patients are male, about 65 years old and the tumor is localized predominantly in the left temporal lobe.
Conclusion: Paired adult familial gbm occurs mainly in the elderly male patient with an integrative diagnosis of primary gbm. Whereas a statistical coincidence seems to be most likely in these rare cases, supplementary and improved genetic studies may identify pathogenetic causes of gbm.
Keywords: multifocal glioblastoma, familial tumor clustering, IDH-1 wildtype, tumor syndrome
This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.Download Article [PDF] View Full Text [HTML][Machine readable]