Chapter

Familial tumour syndromes: neurofibromatosis, schwannomatosis, rhabdoid tumour predisposition, Li–Fraumeni syndrome, Turcot syndrome, Gorlin syndrome, and Cowden syndrome

Scott R. Plotkin, Jaclyn A. Biegel, David Malkin, Robert Martuza and D. Gareth Evans

in Oxford Textbook of Neuro-Oncology

Published on behalf of Oxford University Press

Published in print September 2017 | ISBN: 9780199651870
Published online September 2017 | e-ISBN: 9780191839726 | DOI: https://dx.doi.org/10.1093/med/9780199651870.003.0015

Series: Oxford Textbooks in Clinical Neurology

Familial tumour syndromes: neurofibromatosis, schwannomatosis, rhabdoid tumour predisposition, Li–Fraumeni syndrome, Turcot syndrome, Gorlin syndrome, and Cowden syndrome

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‘Familial tumour syndromes’ reviews some of the genetic syndromes associated with an increased incidence of nervous system tumours, including neurofibromatosis 1, neurofibromatosis 2, schwannomatosis, rhabdoid tumour predisposition, Li–Fraumeni syndrome, Turcot syndrome, Gorlin syndrome, and Cowden syndrome. The chapter reviews the epidemiology of these rare conditions with discussion of current diagnostic criteria. It reviews the genetic basis and pathogenesis of the conditions as well as the availability of genetic testing. It covers the clinical aspects of these conditions, including clinical presentation, associated nervous system tumours, and recommended management of these syndromes with a discussion of the role of imaging. The review is written for the practising neuro-oncologist and other specialists who care for patients with genetic syndromes affecting the nervous system.

Chapter.  15067 words.  Illustrated.

Subjects: Neurology ; Radiation Oncology

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