Subependymal giant cell astrocytoma medical therapy: Difference between revisions

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:*The chemical composition of [[everolimus]] is similar to [[rapamycin]].<ref name="pmid21465222">{{cite journal| author=Campen CJ, Porter BE| title=Subependymal Giant Cell Astrocytoma (SEGA) Treatment Update. | journal=Curr Treat Options Neurol | year= 2011 | volume= 13 | issue= 4 | pages= 380-5 | pmid=21465222 | doi=10.1007/s11940-011-0123-z | pmc=PMC3130084 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21465222  }} </ref>
:*The chemical composition of [[everolimus]] is similar to [[rapamycin]].<ref name="pmid21465222">{{cite journal| author=Campen CJ, Porter BE| title=Subependymal Giant Cell Astrocytoma (SEGA) Treatment Update. | journal=Curr Treat Options Neurol | year= 2011 | volume= 13 | issue= 4 | pages= 380-5 | pmid=21465222 | doi=10.1007/s11940-011-0123-z | pmc=PMC3130084 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21465222  }} </ref>
:*Everolimus has a greater [[bioavailability]] and shorter [[half life]] in comparison to rapamycin.
:*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:
                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. <ref name="pmid23325902">{{cite journal| author=Krueger DA, Care MM, Agricola K, Tudor C, Mays M, Franz DN| title=Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. | journal=Neurology | year= 2013 | volume= 80 | issue= 6 | pages= 574-80 | pmid=23325902 | doi=10.1212/WNL.0b013e3182815428 | pmc=3589289 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23325902  }} </ref>
*The dose of mTORi can be reduced after an initial response with the tumor volume reduction retained. <ref name="pmid23325902">{{cite journal| author=Krueger DA, Care MM, Agricola K, Tudor C, Mays M, Franz DN| title=Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. | journal=Neurology | year= 2013 | volume= 80 | issue= 6 | pages= 574-80 | pmid=23325902 | doi=10.1212/WNL.0b013e3182815428 | pmc=3589289 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23325902  }} </ref>

Revision as of 16:24, 31 October 2019

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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
  • 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
  • Rapamycin may be associated with a decrease in size of the tumor.[3]
  • The standard dosage is 1.5 mg/m2 per day. [1]
  • In adults, this regimen could be used: Rapamycin PO 0.2 mg/kg/day.[3]
  • If the drug is stopped, the tumors may regrow.[4]


  • 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.[5]
  • It may be associated with an improvement in the quality of life and cognition score overtime.[5]
  • 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:
                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. [6]
  • 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.[7]

References

  1. 1.0 1.1 1.2 1.3 1.4 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. 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. 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.
  4. 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.
  5. 5.0 5.1 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.
  6. 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.
  7. 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.


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