Subependymal giant cell astrocytoma medical therapy: Difference between revisions
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:*The goal of [[rapamycin]] ([[sirolimus]]) is to either shrink or stabilize the subependymal giant cell astrocytoma. | :*The goal of [[rapamycin]] ([[sirolimus]]) is to either shrink or stabilize the subependymal giant cell astrocytoma. | ||
:*In adults, this regimen could be used: Rapamycin PO 0.2 mg/kg/day.<ref name="pmid18952591">{{cite journal| author=Koenig MK, Butler IJ, Northrup H| title=Regression of subependymal giant cell astrocytoma with rapamycin in tuberous sclerosis complex. | journal=J Child Neurol | year= 2008 | volume= 23 | issue= 10 | pages= 1238-9 | pmid=18952591 | doi=10.1177/0883073808321764 | pmc=3072698 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18952591 }} </ref> | :*In adults, this regimen could be used: Rapamycin PO 0.2 mg/kg/day.<ref name="pmid18952591">{{cite journal| author=Koenig MK, Butler IJ, Northrup H| title=Regression of subependymal giant cell astrocytoma with rapamycin in tuberous sclerosis complex. | journal=J Child Neurol | year= 2008 | volume= 23 | issue= 10 | pages= 1238-9 | pmid=18952591 | doi=10.1177/0883073808321764 | pmc=3072698 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18952591 }} </ref> | ||
: | :*However, if the drug is stopped, the tumors may regrow.<ref name="pmid16453317">{{cite journal| author=Franz DN, Leonard J, Tudor C, Chuck G, Care M, Sethuraman G et al.| title=Rapamycin causes regression of astrocytomas in tuberous sclerosis complex. | journal=Ann Neurol | year= 2006 | volume= 59 | issue= 3 | pages= 490-8 | pmid=16453317 | doi=10.1002/ana.20784 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16453317 }} </ref> | ||
*Approximately 65% of the tumor mass is reduced by the rapamycin therapy. | *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.<ref name="RothRoach2013">{{cite journal|last1=Roth|first1=Jonathan|last2=Roach|first2=E. Steve|last3=Bartels|first3=Ute|last4=Jóźwiak|first4=Sergiusz|last5=Koenig|first5=Mary Kay|last6=Weiner|first6=Howard L.|last7=Franz|first7=David N.|last8=Wang|first8=Henry Z.|title=Subependymal Giant Cell Astrocytoma: Diagnosis, Screening, and Treatment. Recommendations From the International Tuberous Sclerosis Complex Consensus Conference 2012|journal=Pediatric Neurology|volume=49|issue=6|year=2013|pages=439–444|issn=08878994|doi=10.1016/j.pediatrneurol.2013.08.017}}</ref> | *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.<ref name="RothRoach2013">{{cite journal|last1=Roth|first1=Jonathan|last2=Roach|first2=E. Steve|last3=Bartels|first3=Ute|last4=Jóźwiak|first4=Sergiusz|last5=Koenig|first5=Mary Kay|last6=Weiner|first6=Howard L.|last7=Franz|first7=David N.|last8=Wang|first8=Henry Z.|title=Subependymal Giant Cell Astrocytoma: Diagnosis, Screening, and Treatment. Recommendations From the International Tuberous Sclerosis Complex Consensus Conference 2012|journal=Pediatric Neurology|volume=49|issue=6|year=2013|pages=439–444|issn=08878994|doi=10.1016/j.pediatrneurol.2013.08.017}}</ref> | ||
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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
mTOR inhibitors
- Rapamycin
- Rapamycin may be associated with a decrease in size of the tumor.[3]
- The goal of rapamycin (sirolimus) is to either shrink or stabilize the subependymal giant cell astrocytoma.
- In adults, this regimen could be used: Rapamycin PO 0.2 mg/kg/day.[3]
- However, if the drug is stopped, the tumors may regrow.[4]
- 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.[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]
Chemotherapeutic agents (mTOR inhibitors; mTORi therapy) for subependymal giant cell astrocytoma include rapamycin and everolimus.[1][5]
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
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 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.
- ↑ 5.0 5.1 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.