NDLI: Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex-Current Views on Their Pathogenesis and Management.

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Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex-Current Views on Their Pathogenesis and Management.

Content Provider	Europe PMC
Author	Gao, Chao Zabielska, Bernadeta Jiao, Fuyong Mei, Daoqi Wang, Xiaona Kotulska, Katarzyna Jozwiak, Sergiusz
Editor	Aguglia, Umberto
Copyright Year	2023
Abstract	Introduction, Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder caused by mutations inactivating TSC1 or TSC2 genes and characterized by the presence of tumors involving many organs, including the brain, heart, kidneys, and skin. Subependymal giant cell astrocytoma (SEGA) is a slow-growing brain tumor almost exclusively associated with TSC. State of the art Despite the fact that SEGAs are benign, they require well-considered decisions regarding the timing and modality of pharmacological or surgical treatment. In TSC children and adolescents, SEGA is the major cause of mortality and morbidity. Clinical implications Until recently, surgical resection has been the standard therapy for SEGAs but the discovery of the role of the mTOR pathway and the introduction of mTOR inhibitors to clinical practice changed the therapeutic landscape of these tumors. In the current paper, we discuss the pros and cons of mTOR inhibitors and surgical approaches in SEGA treatment. Future directions In 2021, the International Tuberous Sclerosis Complex Consensus Group proposed a new integrative strategy for SEGA management. In the following review, we discuss the proposed recommendations and report the results of the literature search for the latest treatment directions.
Journal	Journal of Clinical Medicine [J Clin Med]
Volume Number	12
DOI	10.3390/jcm12030956
PubMed Central reference number	PMC9917805
Issue Number	3
PubMed reference number	36769603
e-ISSN	20770383
Language	English
Publisher	Molecular Diversity Preservation International (MDPI)
Publisher Date	2023-01-26
Access Restriction	Open
Subject Keyword	subependymal giant cell astrocytoma (SEGA) tuberous sclerosis complex (TSC) mTOR inhibitors management recommendations
Content Type	Text
Resource Type	Article
Subject	Medicine

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Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex-Current Views on Their Pathogenesis and Management.

State of the art

Clinical implications

Future directions