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Subependymal Giant Cell Astrocytomas (SEGAs): a Model of Targeting Tumor Growth and Epilepsy

  • Neuro-oncology (R Soffietti, Section Editor)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

Subependymal giant cell astrocytomas (SEGAs) are grade I astrocytic tumors according to WHO 2016, mainly associated to tuberous sclerosis complex. They are rare and achievements in their management are often the result of small series and case reports. The main purpose of this review was to summarize recent developments in diagnosis and treatment of SEGAs.

Recent findings

The role of treatment with mTOR inhibitors has been confirmed through many studies in the last decade. New treatment options for patients with contraindication to surgery have been proposed, like Gamma Knife and laser interstitial thermal therapy (LITT). Furthermore, more recent preclinical studies, investigating MAPK/ERK pathways, CK2 protein (casein kinase), and metalloproteinases and their tissue inhibitors (TIMPs), have been published, suggesting new pharmacological treatments.

Summary

This review is focused on natural history, pathogenesis, diagnosis, and treatment of SEGAs, with an update of the more recent developments. SEGAs are mainly associated to tuberous sclerosis complex, while in sporadic forms to mutations in genes TSC1 or TSC2 limited to the tumor. TSC2 mutation leads to higher incidence of SEGAs, earlier onset and more aggressive course. Diagnosis is made by MRI and by criteria defined in the EXIST-1 study. Treatment options are surgery and pharmacological treatment with mTOR inhibitors. LITT shows promising results. Besides tumor grade, SEGAs show a continuous tendency to growth after incomplete resection or withdrawal of mTOR inhibitors. Preclinical studies have explored novel treatment options, but they need to be confirmed in clinical trials.

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Authors and Affiliations

  1. Department of Neuro-Oncology, University and City of Health and Science Hospital, 10126, Turin, Italy

    Francesca Mo MD, Alessia Pellerino MD, PhD & Roberta Rudà MD

  2. Department of Neurology, Castelfranco Veneto and Treviso Hospital, 31100, Treviso, Italy

    Roberta Rudà MD

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  1. Francesca Mo MD
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Correspondence to Roberta Rudà MD.

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Francesca Mo declares no competing interests. Alessia Pellerino declares no competing interests. Roberta Rudà declares no competing interests.

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Mo, F., Pellerino, A. & Rudà, R. Subependymal Giant Cell Astrocytomas (SEGAs): a Model of Targeting Tumor Growth and Epilepsy. Curr Treat Options Neurol 23, 18 (2021). https://doi.org/10.1007/s11940-021-00673-5

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