결절성 경화증 환자에서 경막하 거대세포 성상세포종의감마나이프와 수술치료 1례
A Case of Tuberous Sclerosis with Subependymal Giant Cell Astrocytoma (SEGA) Treated with Gamma Knife Surgery
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초록
저자들은 결절성 경화증에서 흔하게 동반될 수 있는 SEGA가 있는 환자에서 GK-SRS를 시행하여초기 경과가 좋았으나, 재발을 하여 결국 수술적 절제를 시행하게 된 증례를 경험하여 이를 보고하였다.
Abstract
Subependymal giant cell astrocytoma (SEGA) is the most common brain tumor that develops in 5-20% of patients with tuberous sclerosis complex. Although these lesions are slowly growing tumor, they can cause morbidity and mortality due to acute hydrocephalus because of their prevalent location near the foramen of Monro. Surgery has been the standard care for SEGAs demonstrating serial growth and symptomatic hydrocephalus. However, not all SEGAs are treatable by complete surgical resection. Gamma Knife stereotactic radiosurgery can be considered as a second option, but it has shown highly variable responses in patients with limited data. In recent years, drugs with novel mechanism, sirolimus and other mammalian target of rapamycin (mTOR) inhibitors have been found to reduce the size of SEGAs. We report a case of treatment of SEGA with gamma knife surgery which continued for two years without progression, even though surgical resection was done, finally.
참고문헌 (19)
- Jozwiak J, "Possible mechanisms of disease development in tuberous sclerosis", Lancet Oncol, 9, pp.73~79 (2008)
- O'Callaghan FJ, "Prevalence of tuberous sclerosis estimated by capture-recapture analysis", Lancet, 351, pp.1490~ (1998)
- Schwartz RA, "Tuberous sclerosis complex: advances in diagnosis, genetics, and management", J Am Acad Dermatol, 57, pp.189~202 (2007)
- 조형래, "소아기 결절성 경화증에 대한 임상적 고찰 : 새로운 진단 기준에 따른 진단을 중심으로", 대한소아신경학회지, 17(2), pp.174~184 (2009), 대한소아신경학회
- Adriaensen ME, "Prevalence of subependymal giant cell tumors in patients with tuberous sclerosis and a review of the literature", Eur J Neurol, 16, pp.691~696 (2009)
- Ichikawa T, "A case of solitary subependymal giant cell astrocytoma: two somatic hits of TSC2 in the tumor, without evidence of somatic mosaicism", Mol Diagn, 7, pp.544~549 (2005)
- Goh S, "Subependymal giant cell tumors in tuberous sclerosis complex", Neurology, 63, pp.1457~1461 (2004)
- Kwiatkowska J, "Mosaicism in tuberous sclerosis as a potential cause of the failure of molecular diagnosis", N Engl J Med, 340, pp.703~707 (1999)
- Cuccia V, "Subependymal giant cell astrocytoma in children with tuberous sclerosis", Childs Nerv Syst, 19, pp.232~243 (2003)
- Krueger DA, "Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis", N Engl J Med, 363, pp.1801~1811 (2010)
- Crino PB, "New developments in the neurobiology of the tuberous sclerosis complex", Neurology, 53, pp.1384~1390 (1999)
- Jozwiak S, "Management of subependymal giant cell astrocytoma(SEGA)associated with tuberous sclerosis complex(TSC) : Clinical recommendations", Eur J Paediatr Neurol, 17, pp.348~352 (2013)
- Campen CJ, "Subependymal Giant Cell Astrocytoma(SEGA)Treatment Update", Curr Treat Options Neurol, 13, pp.380~385 (2011)
- Cai R, "Combined intra-and extra-endoscopic techniques for aggressive resection of subependymal giant cell astrocytomas", World Neurosurg, 73, pp.713~718 (2010)
- Park KJ, "Gamma Knife surgery for subependymal giant cell astrocytomas. Clinical article", J Neurosurg, 114, pp.808~813 (2011)
- Beaumont TL, "Advances in the management of subependymal giant cell astrocytoma", Childs Nerv Syst, 28, pp.963~968 (2012)
- Matsumura H, "Glioblastoma following radiotherapy in a patient with tuberous sclerosis", Neurol Med Chir (Tokyo), 38, pp.287~291 (1998)
- Orlova KA, "The tuberous sclerosis complex", Ann N Y Acad Sci, 1184, pp.87~105 (2010)
- Franz DN, "Rapamycin causes regression of astrocytomas in tuberous sclerosis complex", Ann Neurol, 59, pp.490~498 (2006)