결절성 경화증(Tuberoussclerosis)환자에서 뇌실막밑 거대세포 별아교세포종의(Subependymalgiantcellastrocytoma)치료
Management of Subependymal Giant Cell Astrocytomas in Patients with Tuberous Sclerosis
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목적: 결절성 경화증에서 뇌실막밑 거대세포 별아교세포종은 잘알려져 있는 뇌 내 병변중의 하나로서, 서서히 증식하지만, 몬로공의폐쇄를 유발하여 뇌압상승으로 인한 신경학적 합병증을 유발하기도한다. 최적요법은 수술적인 종양 절제술이지만, 합병증과 위험성으로인하여 수술의 시기에 있어 많은 논란이 있어왔다. 최근 mTOR 억제제의 개발로 약물치료에 대한 연구도 이루어져 왔으나, 아직은 그 근거가 충분치 않다. 이에 세브란스 어린이 병원에서 치료받은 환자들을대상으로 뇌실막밑 거대세포 별아교세포종의 치료 현황 및 수술 결과를 분석하여 치료에 도움이 되고자 한다. 방법: 본원에서 2007년부터 2014년까지 진단받은 161명의 결절성경화증 환자들을 후향적으로 분석하였다. 결과: 17명의 뇌실막밑 거대세포 별아교세포종 환자가 있었으며 이중 네 명에서 수술적인 절제술을 받았다. 네 명 중 뇌압상승 증상이있었던 두 명의 환자에서는 신경학적 합병증이 발생하였고, 영구적인뇌실 복강 션트를 삽입하였으나, 증상이 없었던 두 명의 환자에서는종양의 전 절제술이 가능하였으며 신경학적인 합병증도 없었다. 결론: 이를 통하여 뇌실막밑 거대세포 별아교세포종의 치료로서약물 치료 방법뿐 아니라 조기 수술적인 치료가 증상이 없는 환자에서도 반드시 고려되어야 한다고 판단된다.
Abstract
Purpose: Subependymal giant cell astrocytomas (SEGAs) are a well-known complication of tuberous sclerosis complex (TSC). Treatment experience has been accumulated for several years. The aim of this study was to review our experiences in treatment of SEGAs and prognosis. Method: From March 2007 to March 2014, 161 TSC patients were diagnosed. Of 161 patients, 17 patients met diagnostic criteria of SEGAs. We reviewed clinical data of SEGA patients retrogradely. Results: Among 17 SEGAs patients, five patients underwent surgical treatment. Of five patients who underwent surgery, including two patients who underwent gamma knife surgery before tumorectomy, four patients underwent tumorectomy, and one patient underwent shunt operation. Out of four patients who underwent surgery, two patients with increased intracranial pressure (IICP) symptoms before surgery showed worse neurological outcome than the other two patients without IICP symptoms. Conclusion: Our experiences suggest that early surgical intervention should be considered as one of treatment modalities for asymptomatic SEGA patients.
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