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CC BY 4.0 · J Neurol Surg Rep 2025; 86(01): e31-e35
DOI: 10.1055/a-2530-5965
Review Report

Subependymal Giant Cell Astrocytoma in an Adult without Tuberous Sclerosis: Systematic Review and Illustrative Case Example

Brandon M. Holler
1   Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States
,
Alexander R. Evans 
1   Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States
,
Abigail York
1   Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States
,
Christopher S. Graffeo
1   Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States
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Abstract

Background Subependymal giant cell astrocytoma (SEGA) is a rare neoplasm arising from subependymal tissue. Predominantly associated with the tuberous sclerosis complex (TSC), SEGA may present with a range of diverse symptoms, most commonly seizures or neurocutaneous features of TSC. We present a novel case of sporadic SEGA in a 59-year-old woman who presented with acute intraparenchymal hemorrhage (IPH).

Methods Systematic literature review and illustrative case example.

Results A 59-year-old woman presented with a headache decreased level of consciousness, and acute IPH involving the anterior septum pellucidum and right medial caudate head. MRI was concerning for an underlying neoplasm, which grew slowly on follow-up imaging, prompting microsurgical resection. A gross total resection was achieved, and postoperative pathology confirmed SEGA (WHO grade I) without TSC1/2 mutation. She remained disease-free and neurologically intact at 1-year follow-up. A systematic review identified seven publications that revealed pathologically confirmed SEGA in nine adult patients without TSC. Headache, papilledema, and visual disturbances were the most common presenting symptoms. Treatment protocols included microsurgical resection versus biopsy followed by radiographic surveillance, and the overall rate of symptom-free survival was at least 80% as of the last follow-up.

Conclusion We report the tenth case of sporadic SEGA in an adult patient without TSC, as well as an associated systematic review of this rare neoplastic entity. Further study is required to identify risk factors for the development of sporadic SEGA, as well as potential avenues for the management of this disease that may depart from the standard protocol in pediatric TSC patients.

Keywords

subependymal giant cell astrocytoma - tuberous sclerosis - systematic review


Publication History

Received: 24 September 2024

Accepted: 27 December 2024

Accepted Manuscript online:
03 February 2025

Article published online:
27 February 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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