Issue release date: 12.11.2024
2Erciyes University, Faculty of Medicine, Department of Pediatric Neurology, Kayseri, Turkey
3Osmangazi University, Faculty of Medicine, Department of Pediatric Neurology, Eskisehir, Turkey
4Erciyes University, Faculty of Medicine, Department of Pathology, Kayseri, Turkey
5Erciyes University, Faculty of Medicine, Department of Neurosurgery, Kayseri, Turkey DOI : 10.5137/1019-5149.JTN.4169-11.0 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.
Keywords : Tuberous sclerosis complex, Subependymal giant cell astrositoma, Surgical timing