Published in:
01-04-2014 | Review Paper
RETRACTED ARTICLE: Subependymal giant cell astrocytoma: current
concepts, management, and future directions
Authors:
Taohui Ouyang, Na Zhang, Thomas Benjamin, Long Wang, Jiantong Jiao, Yiqing Zhao, Jian Chen
Published in:
Child's Nervous System
|
Issue 4/2014
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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.