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

Gao, Chao; Zabielska, Bernadeta; Jiao, Fuyong; Mei, Daoqi; Wang, Xiaona; Kotulska, Katarzyna; Jozwiak, Sergiusz

doi:10.3390/jcm12030956

Open AccessReview

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

by

Chao Gao

¹

,

Bernadeta Zabielska

²,

Fuyong Jiao

³,

Daoqi Mei

⁴,

Xiaona Wang

⁵,

Katarzyna Kotulska

⁶ and

Sergiusz Jozwiak

^2,7,*

¹

Department of Rehabilitation Medicine, Henan Children’s Hospital, Children’s Hospital Affiliated to Zhengzhou University, Zhengzhou 450018, China

²

Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland

³

Pediatric Neurology Section, Children’s Hospital of Shaanxi Provincial People’s Hospital, Xi’an 710068, China

⁴

Department of Neurology, Henan Children’s Hospital, Children’s Hospital Affiliated to Zhengzhou University, Zhengzhou 450018, China

⁵

Henan Key Laboratory of Children’s Genetics and Metabolic Diseases, Henan Children’s Hospital, Children’s Hospital Affiliated to Zhengzhou University, Zhengzhou 450018, China

⁶

Department of Neurology and Epileptology, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland

⁷

Research Department, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland

^*

Author to whom correspondence should be addressed.

J. Clin. Med. 2023, 12(3), 956; https://doi.org/10.3390/jcm12030956

Submission received: 28 November 2022 / Revised: 21 January 2023 / Accepted: 22 January 2023 / Published: 26 January 2023

(This article belongs to the Section Clinical Neurology)

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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.

Keywords: subependymal giant cell astrocytoma (SEGA); tuberous sclerosis complex (TSC); mTOR inhibitors; management; recommendations

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MDPI and ACS Style

Gao, C.; Zabielska, B.; Jiao, F.; Mei, D.; Wang, X.; Kotulska, K.; Jozwiak, S. Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex—Current Views on Their Pathogenesis and Management. J. Clin. Med. 2023, 12, 956. https://doi.org/10.3390/jcm12030956

AMA Style

Gao C, Zabielska B, Jiao F, Mei D, Wang X, Kotulska K, Jozwiak S. Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex—Current Views on Their Pathogenesis and Management. Journal of Clinical Medicine. 2023; 12(3):956. https://doi.org/10.3390/jcm12030956

Chicago/Turabian Style

Gao, Chao, Bernadeta Zabielska, Fuyong Jiao, Daoqi Mei, Xiaona Wang, Katarzyna Kotulska, and Sergiusz Jozwiak. 2023. "Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex—Current Views on Their Pathogenesis and Management" Journal of Clinical Medicine 12, no. 3: 956. https://doi.org/10.3390/jcm12030956

APA Style

Gao, C., Zabielska, B., Jiao, F., Mei, D., Wang, X., Kotulska, K., & Jozwiak, S. (2023). Subependymal Giant Cell Astrocytomas in Tuberous Sclerosis Complex—Current Views on Their Pathogenesis and Management. Journal of Clinical Medicine, 12(3), 956. https://doi.org/10.3390/jcm12030956

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

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