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
- The mainstay treatment of subependymal giant cell astrocytoma is surgical resection but medical therapy may be used in certain cases such as:
- Bilaterally located subependymal giant cell astrocytomas
- Invasive lesions to the neighboring structures
- Growing residual tumors
- Lesions unlikely to be treated with gross total resection
- Multiple lesions
mTOR inhibitors
- Rapamycin
- 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.[5]
- 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.
- Everolimus
- Everolimus was approved for the treatment of subependymal giant cell astrocytoma by the US Food and Drug Administration (FDA), in 2012.[6]
- Everolimus may be associated with marked volume reduction of the tumor and a reduction in the frequency of seizures. The reduction in the primary tumor is more rapid during the first three months of treatment.[7]
- It may be associated with an improvement in the quality of life and cognition score overtime.[7]
- The chemical composition of everolimus is similar to rapamycin.[1]
- Everolimus has a greater bioavailability and shorter half life in comparison to rapamycin.
- The dose of mTORi can be reduced after an initial response with the tumor volume reduction retained. [8]
- Stomatitis and upper respiratory infections are the most common adverse effects of mTOR inhibitors. Other adverse effects include bronchitis, leukopenia, vomiting, and elevation of total cholesterol, LDL, and triglycerides.[9]
References
- ↑ 1.0 1.1 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 Koenig MK, Butler IJ, Northrup H (2008). "Regression of subependymal giant cell astrocytoma with rapamycin in tuberous sclerosis complex". J Child Neurol. 23 (10): 1238–9. doi:10.1177/0883073808321764. PMC 3072698. PMID 18952591.
- ↑ Franz DN, Leonard J, Tudor C, Chuck G, Care M, Sethuraman G; et al. (2006). "Rapamycin causes regression of astrocytomas in tuberous sclerosis complex". Ann Neurol. 59 (3): 490–8. doi:10.1002/ana.20784. PMID 16453317.
- ↑ 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.
- ↑ FDA Approval for Everolimus. National cancer institute 2015. http://www.cancer.gov/about-cancer/treatment/drugs/fda-everolimus. Accessed on November 5, 2015
- ↑ 7.0 7.1 Krueger, Darcy A.; Care, Marguerite M.; Holland, Katherine; Agricola, Karen; Tudor, Cynthia; Mangeshkar, Prajakta; Wilson, Kimberly A.; Byars, Anna; Sahmoud, Tarek; Franz, David Neal (2010). "Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous Sclerosis". New England Journal of Medicine. 363 (19): 1801–1811. doi:10.1056/NEJMoa1001671. ISSN 0028-4793.
- ↑ Krueger DA, Care MM, Agricola K, Tudor C, Mays M, Franz DN (2013). "Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma". Neurology. 80 (6): 574–80. doi:10.1212/WNL.0b013e3182815428. PMC 3589289. PMID 23325902.
- ↑ Aguilera D, Flamini R, Mazewski C, Schniederjan M, Hayes L, Boydston W; et al. (2014). "Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus". J Pediatr Hematol Oncol. 36 (7): e448–51. doi:10.1097/MPH.0000000000000005. PMC 4009394. PMID 24276039.