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 mainstay therapy for subependymal giant cell astrocytoma is surgery, but medical therapy is preferred in some cases.
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
- The goal of medical therapy is to shrink or stabilize the tumor.[1]
- Contraindications to treating subependymal giant cell astrocytoma with medical therapy include:[1]
- The tumors that cause significant hydrocephalus with impending herniation
- Patients with severe acute infections
mTOR inhibitors
- Rapamycin
- Everolimus
- 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.[4]
- It may be associated with an improvement in the quality of life and cognition score overtime.[4]
- The chemical composition of everolimus is similar to rapamycin.[1]
- Everolimus has a greater bioavailability and shorter half life in comparison to rapamycin.
- The dosing of everolimus depends on the body surface area of the patient:[1]
0.5 m2 to 1.2 m2: 2.5 mg once daily
1.3 m2 to 2.1 m2: 5 mg once daily
>2.2 m2: 7.5 mg once daily
- The dose of mTORi can be reduced after an initial response with the tumor volume reduction retained. [5]
- Stomatitis and upper respiratory infections are the most common adverse effects of mTOR inhibitors. Other adverse effects include bronchitis, leukopenia, vomiting, thrombocytopenia, acneiform rash, hypercholesterolemia, immunosuppression, and impaired wound healing.[6][1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 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.
- ↑ 2.0 2.1 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.
- ↑ 4.0 4.1 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.