Abstract
Background
Subependymal giant cell astrocytoma (SEGA) is the most common central nervous system tumor in patients with tuberous sclerosis complex (TSC). SEGAs are generally benign, non-infiltrative lesions, but they can lead to intracranial hypertension, obstructive hydrocephalus, focal neurologic deficits, and even sudden death.
Discussion
Surgical resection has been the standard treatment for SEGAs, and it is generally curative with complete resection. However, not all SEGAs are amenable to safe and complete resection. Gamma Knife stereotactic radiosurgery provides another treatment option as a primary or adjuvant treatment for SEGAs, but it has highly variable response effects with sporadic cases demonstrating its efficacy. Recently, biologically targeted pharmacotherapy with mammalian target of rapamycin (mTOR) inhibitors such as sirolimus and everolimus has provided a safe and efficacious treatment option for patients with SEGAs. However, SEGAs can recur few months after drug discontinuation, indicating that mTOR inhibitors may need to be continued to avoid recurrence. Further studies are needed to evaluate the advantages and adverse effects of long-term treatment with mTOR inhibitors. This review presents an overview of the current knowledge and particularly highlights the surgical and medical options of SEGAs in patients with TSC.
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Abbreviations
- SEGA:
-
subependymal giant cell astrocytoma
- TSC:
-
tuberous sclerosis complex
- GKS:
-
Gamma Knife stereotactic radiosurgery
- mTOR:
-
mammalian target of rapamycin
- SEN:
-
subependymal nodules
- LOH:
-
loss of heterozygosity
- ERK:
-
extracellular signal-regulated kinase
- DAPK:
-
death-associated protein kinase
- AEDs:
-
antiepileptic drugs
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Thanks are given for the important recommendations made by Jian Chen’s colleagues on this article.
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Taohui Ouyang and Na Zhang contributed equally to this work.
This article has been retracted by the Journal Editors due to considerable overlap with the article “Advances in the management of subependymal giant cell astrocytoma” by Thomas L. Beaumont, David D. Limbrick, Matthew D. Smyth published in the same journal, Volume 28, no. 7, 2012 pp 963-968.
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Ouyang, T., Zhang, N., Benjamin, T. et al. RETRACTED ARTICLE: Subependymal giant cell astrocytoma: current concepts, management, and future directions. Childs Nerv Syst 30, 561–570 (2014). https://doi.org/10.1007/s00381-014-2383-x
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DOI: https://doi.org/10.1007/s00381-014-2383-x