TY - JOUR
T1 - Advances in the management of subependymal giant cell astrocytoma
AU - Beaumont, Thomas L.
AU - Limbrick, David D.
AU - Smyth, Matthew D.
N1 - Funding Information:
Disclosures Editorial support was provided by ApotheCom, which has been funded by Novartis Pharmaceuticals, Inc.
PY - 2012/7
Y1 - 2012/7
N2 - Background: Subependymal giant cell astrocytoma (SEGA) is the most common central nervous system tumor in patients with tuberous sclerosis complex (TSC). Although these lesions are generally benign and non-infiltrative, they commonly arise in the region of the foramen of Monro, where they can cause obstructive hydrocephalus and sudden death. Methods: Surgical resection has been, and presently remains, the standard treatment for SEGAs demonstrating serial growth on neuroimaging in the setting of symptomatic hydrocephalus or progressive ventriculomegaly. Discussion Surgery can be curative; however, not all SEGAs are amenable to safe and complete resection. Gamma Knife stereotactic radiosurgery provides another treatment option but has highly variable response rates with limited data demonstrating its efficacy. Newer medical therapy targeting mammalian target of rapamycin (mTOR), the key protein kinase that is constitutively activated in TSC, has demonstrated promising results in recent clinical trials. In both case reports and clinical trials, treatment with mTOR inhibitors results in a significant reduction in SEGA volume and improvement or resolution of ventriculomegaly. This has led to the approval of everolimus for the treatment of SEGA in tuberous sclerosis patients who are not candidates for surgery. This review summarizes the surgical and medical management of SEGA in patients with TSC.
AB - Background: Subependymal giant cell astrocytoma (SEGA) is the most common central nervous system tumor in patients with tuberous sclerosis complex (TSC). Although these lesions are generally benign and non-infiltrative, they commonly arise in the region of the foramen of Monro, where they can cause obstructive hydrocephalus and sudden death. Methods: Surgical resection has been, and presently remains, the standard treatment for SEGAs demonstrating serial growth on neuroimaging in the setting of symptomatic hydrocephalus or progressive ventriculomegaly. Discussion Surgery can be curative; however, not all SEGAs are amenable to safe and complete resection. Gamma Knife stereotactic radiosurgery provides another treatment option but has highly variable response rates with limited data demonstrating its efficacy. Newer medical therapy targeting mammalian target of rapamycin (mTOR), the key protein kinase that is constitutively activated in TSC, has demonstrated promising results in recent clinical trials. In both case reports and clinical trials, treatment with mTOR inhibitors results in a significant reduction in SEGA volume and improvement or resolution of ventriculomegaly. This has led to the approval of everolimus for the treatment of SEGA in tuberous sclerosis patients who are not candidates for surgery. This review summarizes the surgical and medical management of SEGA in patients with TSC.
KW - Everolimus
KW - Sirolimus
KW - Subependymal giant cell astrocytoma
KW - Tuberous sclerosis complex
UR - http://www.scopus.com/inward/record.url?scp=84863726665&partnerID=8YFLogxK
U2 - 10.1007/s00381-012-1785-x
DO - 10.1007/s00381-012-1785-x
M3 - Article
C2 - 22562196
AN - SCOPUS:84863726665
SN - 0256-7040
VL - 28
SP - 963
EP - 968
JO - Child's Nervous System
JF - Child's Nervous System
IS - 7
ER -