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Recent developments in brain tumor predisposing syndromes
Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
Umeå University, Faculty of Medicine, Department of Radiation Sciences, Oncology.
2016 (English)In: Acta Oncologica, ISSN 0284-186X, E-ISSN 1651-226X, Vol. 55, no 4, 401-411 p.Article, review/survey (Refereed) PublishedText
Abstract [en]

The etiologies of brain tumors are in the most cases unknown, but improvements in genetics and DNA screening have helped to identify a wide range of brain tumor predisposition disorders. In this review we are discussing some of the most common predisposition disorders, namely: neurofibromatosis type 1 and 2, schwannomatosis, rhabdoid tumor predisposition disorder, nevoid basal cell carcinoma syndrome (Gorlin), tuberous sclerosis complex, von Hippel-Lindau, Li-Fraumeni and Turcot syndromes. Recent findings from the GLIOGENE collaboration and the newly identified glioma causing gene POT1, will also be discussed. Genetics. We will describe these disorders from a genetic and clinical standpoint, focusing on the difference in clinical symptoms depending on the underlying gene or germline mutation. Central nervous system (CNS) tumors. Most of these disorders predispose the carriers to a wide range of symptoms. Herein, we will focus particularly on tumors affecting the CNS and discuss improvements of targeted therapy for the particular disorders.

Place, publisher, year, edition, pages
Taylor & Francis, 2016. Vol. 55, no 4, 401-411 p.
National Category
Cancer and Oncology
Identifiers
URN: urn:nbn:se:umu:diva-119059DOI: 10.3109/0284186X.2015.1107190ISI: 000372125400001PubMedID: 26634384OAI: oai:DiVA.org:umu-119059DiVA: diva2:922183
Available from: 2016-04-22 Created: 2016-04-11 Last updated: 2016-04-22Bibliographically approved

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Johansson, GunnarAndersson, UlrikaMelin, Beatrice
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