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Subependymal Giant Cell Astrocytoma: Role of mTOR Pathway and Its Inhibitors

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Tumors of the Central Nervous System, Volume 5

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 5))

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Abstract

Subependymal giant cell astrocytoma is a distinct brain tumor growing near the foramen of Monro. Although histologically benign, these tumors may produce hydrocephalus and are associated with significant morbidity and mortality. Until recently, the only recommended treatment for these tumors was surgical resection. Subependymal giant cell astrocytoma is almost exclusively associated with Tuberous Sclerosis Complex. Tuberous Sclerosis Complex is a relatively frequent neurocutaneous disease, caused by the mutation in either of two genes: TSC1 or TSC2. Both genes act as tumor suppressors, and their products were shown to inhibit mTOR pathway. Recently, the recognition of the role of mTOR pathway in the pathogenesis of Tuberous Sclerosis Complex and subependymal giant cell astrocytoma led to clinical trials of mTOR inhibitors. Everolimus was shown to reduce the size of subependymal giant cell astrocytoma and is currently approved by FDA to treat patients with SEGA associated with TSC, who cannot be treated with surgery.

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Authors and Affiliations

  1. Klinika Neurologii I Epileptologii, Instytut Pomnik “Centrum Zdrowia Dziecka”, 04-730, Warszawa, Poland

    Katarzyna Kotulska & Sergiusz Jóźwiak

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  1. Katarzyna Kotulska
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  2. Sergiusz Jóźwiak
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Correspondence to Katarzyna Kotulska .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Kotulska, K., Jóźwiak, S. (2012). Subependymal Giant Cell Astrocytoma: Role of mTOR Pathway and Its Inhibitors. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 5. Tumors of the Central Nervous System, vol 5. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2019-0_5

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  • DOI: https://doi.org/10.1007/978-94-007-2019-0_5

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  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-007-2018-3

  • Online ISBN: 978-94-007-2019-0

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