Surgical Timing of the Subependymal Giant Cell Astrocytoma (SEGA) with the Patients of Tuberous Sclerosis Complex

被引:16
作者
Ekici, Mehmet Ali [1 ]
Kumandas, Sefer [2 ]
Per, Huseyin [2 ]
Ekici, Arzu [3 ]
Tucer, Bulent [5 ]
Gumus, Hakan [2 ]
Canoz, Ozlem [4 ]
Kurtsoy, Ali [5 ]
机构
[1] Bozok Univ, Fac Med, Dept Neurosurg, Yozgat, Turkey
[2] Erciyes Univ, Fac Med, Dept Pediat Neurol, Kayseri, Turkey
[3] Osmangazi Univ, Fac Med, Dept Pediat Neurol, Eskisehir, Turkey
[4] Erciyes Univ, Fac Med, Dept Pathol, Kayseri, Turkey
[5] Erciyes Univ, Fac Med, Dept Neurosurg, Kayseri, Turkey
关键词
Tuberous sclerosis complex; Subependymal giant cell astrositoma; Surgical timing; EARLY-DIAGNOSIS; CHILDREN; MANAGEMENT; SEVERITY; FEATURES; NODULES; DISEASE; TUMORS; TSC2;
D O I
10.5137/1019-5149.JTN.4169-11.0
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:315 / 324
页数:10
相关论文
共 37 条
[1]  
Baron Y, 1999, AM J NEURORADIOL, V20, P907
[2]   Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon's perspective [J].
Berhouma, Moncef .
WORLD JOURNAL OF PEDIATRICS, 2010, 6 (02) :103-110
[3]   MR IMAGING OF TUBEROUS SCLEROSIS - PATHOGENESIS OF THIS PHAKOMATOSIS, USE OF GADOPENTETATE DIMEGLUMINE, AND LITERATURE-REVIEW [J].
BRAFFMAN, BH ;
BILANIUK, LT ;
NAIDICH, TP ;
ALTMAN, NR ;
POST, MJD ;
QUENCER, RM ;
ZIMMERMAN, RA ;
BRODY, BA .
RADIOLOGY, 1992, 183 (01) :227-238
[4]   Neuroimaging correlation with neurological severity in tuberous sclerosis complex [J].
Chou, I-Jun ;
Lin, Kuang-Lin ;
Wong, Alex M. ;
Wang, Huei-Shyong ;
Chou, Min-Liang ;
Hung, Po-Cheng ;
Hsieh, Meng-Ying ;
Chang, Mingy Yu .
EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY, 2008, 12 (02) :108-112
[5]   SUBEPENDYMAL GIANT-CELL ASTROCYTOMA IN CHILDREN - AN UNUSUAL DISCREPANCY BETWEEN HISTOLOGICAL AND CLINICAL-FEATURES [J].
CHOW, CW ;
KLUG, GL ;
LEWIS, EA .
JOURNAL OF NEUROSURGERY, 1988, 68 (06) :880-883
[6]   Subependymal giant cell astrocytoma in children with tuberous sclerosis [J].
Cuccia, V ;
Zuccaro, G ;
Sosa, F ;
Monges, J ;
Lubienieky, F ;
Taratuto, AL .
CHILDS NERVOUS SYSTEM, 2003, 19 (04) :232-243
[7]   Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs [J].
Dabora, SL ;
Jozwiak, S ;
Franz, DN ;
Roberts, PS ;
Nieto, A ;
Chung, J ;
Choy, YS ;
Reeve, MP ;
Thiele, E ;
Egelhoff, JC ;
Kasprzyk-Obara, J ;
Domanska-Pakiela, D ;
Kwiatkowski, DJ .
AMERICAN JOURNAL OF HUMAN GENETICS, 2001, 68 (01) :64-80
[8]   Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease:: When should we operate? [J].
de Ribaupierre, Sandrine ;
Dorfmuller, Georg ;
Bulteau, Christine ;
Fohlen, Martine ;
Pinard, Jean-Marc ;
Chiron, Catherine ;
Delalande, Olivier .
NEUROSURGERY, 2007, 60 (01) :83-89
[9]   Brain abnormalities in tuberous sclerosis complex [J].
DiMario, FJ .
JOURNAL OF CHILD NEUROLOGY, 2004, 19 (09) :650-657
[10]   Cerebellar lesions in tuberous sclerosis complex: Neurobehavioral and neuroimaging correlates [J].
Eluvathingal, Thomas J. ;
Behen, Michael E. ;
Chugani, Harry T. ;
Janisse, James ;
Bernardi, Bruno ;
Chakraborty, Pulak ;
Juhasz, Csaba ;
Muzik, Otto ;
Chugani, Diane C. .
JOURNAL OF CHILD NEUROLOGY, 2006, 21 (10) :846-851