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Subependymal Giant Cell Astrocytoma: Treatment

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Tumors of the Central Nervous System, Volume 9

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 9))

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Abstract

Tuberous sclerosis complex (TSC), a ­relatively common genetic disorder, is associated with inactivation of tumor suppressor genes. Hence, tumors of many organ systems are seen in patients with TSC, and with subependymal giant cell astrocytomas (SEGA) seen in 5–20%. SEGAs are low-grade mixed glial/neuronal intracranial tumors arising from the subependymal lining of the ventricles. The primary complication from SEGAs is obstructive hydrocephalus, with sequelae including headache, vision impairment, ­diplopia, and brainstem herniation. Because of their progressive course, and potentially fatal complications, SEGAs usually require treatment. Standard of care has been surgical resection, with low rates of regrowth; making surgical resection a successful and permanent therapeutic strategy. In addition to surgical resection of SEGAs there are now other treatment options including pharmacotherapy and radiotherapy. In case series, sirolimus and everolimus, inhibitors of the mammalian ­target of rapamycin (mTOR), suppressed the growth, and in some cases shrank the size of SEGAs.

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Correspondence to Cynthia J. Campen .

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Campen, C.J. (2012). Subependymal Giant Cell Astrocytoma: Treatment. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 9. Tumors of the Central Nervous System, vol 9. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5488-1_21

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  • DOI: https://doi.org/10.1007/978-94-007-5488-1_21

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  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-007-5487-4

  • Online ISBN: 978-94-007-5488-1

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