Subependymal giant cell astrocytoma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
The predominant therapy for subependymal giant cell astrocytoma is surgical resection. Adjunctive chemotherapy may be required.[1][2]
Medical Therapy
Chemotherapy
mTOR inhibitors such as rapamycin and everolimus may be used in the treatment of subependymal giant cell astrocytoma. Chemotherapeutic agents (mTOR inhibitors; mTORi therapy) for subependymal giant cell astrocytoma include rapamycin and everolimus.[1][3]
Indications
- Lesion(s) for which gross total resection is unlikely
- Invading to neighbouring structures (fornix, hypothalamus, basal ganglia, or genu of internal capsule)
- Deeply seated tumor
- Large-sized tumors (higher bleeding risk with surgery)
- Recurrent tumors
- Multiple, bilateral tumors
Contraindications
- Significant hydrocephalus and impending herniation
- Severe infections
Adverse effects
Rapamycin
- The goal of rapamycin (sirolimus) is to either shrink or stabilize the subependymal giant cell astrocytoma.
- Approximately 65% of the tumor mass is reduced by the rapamycin therapy.
- Patients from the initial report of rapamycin for subependymal giant cell astrocytoma have been receiving this agent for in excess of 10 years with acceptable adverse events.[3]
- It may be possible to reduce the dose of rapamycin after an initial response with preservation of tumor volume reduction.
- Subependymal giant cell astrocytoma growth during the rapamycin therapy is extremely uncommon and most of the individuals who exhibit such growth have remained asymptomatic.
- However, if the drug is stopped, the tumors may regrow.[4]
Everolimus
- Everolimus was approved for the treatment of subependymal giant cell astrocytoma by the US Food and Drug Administration (FDA), in 2012.[5]
- The mTOR inhibitor everolimus may significantly decrease the volume (>50%) of subependymal giant cell astrocytoma at 6 months of treatment.[3]
- The chemical composition of everolimus is similar to rapamycin.[1]
- Everolimus has a greater bioavailability and shorter half life in comparison to rapamycin.
References
- ↑ 1.0 1.1 1.2 Campen CJ, Porter BE (2011). "Subependymal Giant Cell Astrocytoma (SEGA) Treatment Update". Curr Treat Options Neurol. 13 (4): 380–5. doi:10.1007/s11940-011-0123-z. PMC 3130084. PMID 21465222.
- ↑ Jóźwiak S, Nabbout R, Curatolo P, participants of the TSC Consensus Meeting for SEGA and Epilepsy Management (2013). "Management of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex (TSC): Clinical recommendations". Eur J Paediatr Neurol. 17 (4): 348–52. doi:10.1016/j.ejpn.2012.12.008. PMID 23391693.
- ↑ 3.0 3.1 3.2 Roth, Jonathan; Roach, E. Steve; Bartels, Ute; Jóźwiak, Sergiusz; Koenig, Mary Kay; Weiner, Howard L.; Franz, David N.; Wang, Henry Z. (2013). "Subependymal Giant Cell Astrocytoma: Diagnosis, Screening, and Treatment. Recommendations From the International Tuberous Sclerosis Complex Consensus Conference 2012". Pediatric Neurology. 49 (6): 439–444. doi:10.1016/j.pediatrneurol.2013.08.017. ISSN 0887-8994.
- ↑ Pharmacotherapy treatment of subependymal giant cell astrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Subependymal_giant_cell_astrocytoma. Accessed on November 5, 2015
- ↑ FDA Approval for Everolimus. National cancer institute 2015. http://www.cancer.gov/about-cancer/treatment/drugs/fda-everolimus. Accessed on November 5, 2015