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Journal of the Korean Child Neurology Society 2013;21(3):195-199.
Published online September 30, 2013.
A Case of Tuberous Sclerosis with Subependymal Giant Cell Astrocytoma (SEGA) Treated with Gamma Knife Surgery.
Mo Kyung Jung, Sang Mi Lee, Rita Yu, Heung Dong Kim, Joon Soo Lee, Dong Suk Kim, Hoon Chul Kang
1Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea. hipo0207@yuhs.ac
2Department of Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.
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.
Key Words: Tuberous sclerosis, Subependymal giant cell astrocytoma (SEGA), Gamma Knife Radiosurgery


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