AI Summary & Key Takeaways
AI InsightThe early surgical menagement for subependimal giant cell astrocytoma are recommended, and also periodic monitoring even for asymptomatic patients with subependymal nodules.
📌 3 Key Takeaways
Abstract
AIM: Tuberous sclerosis complex has shown a wide variety of clinical, pathologic and radiologic manifestations. Many tumor types are found in tuberous sclerosis, which includes subependimal giant cell astrositoma. The aim of this study is to focus on surgical timing of the tumor. MATERIAL and METHODS: This study included 37 children with tuberous sclerosis presenting to Erciyes Univercity Medical School, whose hospital record were retrospectively evaluated between 1995 and 2010. Of the 5 patients had diagnosed with the subependymal giant cell astrocytoma and three patients were opereted on. RESULTS: In the 27 of the patients had subependimal nodules (73%), cortical tubers were in the 19 patients (51,4%), giant cell astrositoma (SEGA) were in the 5 patients (13,5%). Mental retardation in different level was detected in the 18 patients (48.6%). The other clinical findings of the patients were angiomyolipomas (37.8%), hypomelanotic macules (91.9%), Convulsion (54.1%), adenoma sebaceum (32.4%) , West syndrome (16.22%), shagreeen patch (16.2%), intracardiac tumor (37.8%), subungual fibroma (2.7%), fibroadenom in the neck (2.7%). CONCLUSION: A multidisciplinary approach is essential for an early, accurate diagnosis and proper management of affected individuals. The early surgical menagement for subependimal giant cell astrocytoma are recommended, and also periodic monitoring even for asymptomatic patients with subependymal nodules.
Özet
AMAÇ: Tüberus sklerozis kompleks klinik, patolojik ve radyolojikolarak geniş bir çeşitlilik göstermektedir. Subependimal dev hücreli astrositomanın dahil olduğu birçok tümör tipi bulunur. Bu çalışmanın amacı tümörün cerrahi zamanlamasına dikkat çekmek. YÖNTEM ve GEREÇ: Erciyes Üniversitesi Tıp Fakültesi Hastanesi’nde 1995-2010 yılları arasında hasta kayıtları geriye dönük olarak değerlendirildi ve 37 tüberus sklerozisli hasta çalışmaya dahil edildi. Hastaların 5’ine subependimal dev hücreli astrositom tanısı konuldu ve üç hasta ameliyat edildi. BULGULAR: 27 hastada (%73) subependimal nodul, 19 hastada (%51.4) kortikal tuber, 5 hastada (%13.5) dev hücreli astrositom vardı. 18 hastada (%48.6) farklı seviyelerde mental retardasyon tespit edildi. Hastaların diğer klinik bulguları; anjiyomyolipom (%37.8), hipomelanotik makul (%91.9), konvulzyon (%54.1), adenoma sebaseum (%32.4), west sendromu (%16.2), shagreen lekeleri (%16.2), intrakardiak tümör (%37.8), subungual fibroma (%2.7), boyunda fibro adenomdu (%2.7). SONUÇ: Etkilenmiş bireylerin erken, doğru ve uygun tedavisi için multidisipliner yaklaşım esastır. Subependimal dev hücreli astrositomada erken cerrahi önerilir ve de subependimal nodullü hastalar için peryodik izleme önerilir.