室管膜下巨细胞星形细胞瘤伴发结节性硬化的临床病理观察

Clinicopathologic study of subependymal giant cell astrocytoma associated with tuberous sclerosis

目的：探讨室管膜下巨细胞星形细胞瘤（subependymal giant—cellastrocytoma，SEGA）的临床病理特征、免疫表型特征及其与预后的关系。方法：对5例SEGA的临床特点，组织学特点及免疫组化标记进行分析，并对所有患者进行随访。结果：5例SEGA患者均为青少年主要症状为头痛、呕吐。肿瘤均位于侧脑室，伴有结节性硬化。病理形态学特征由大的圆形或多角形“节细胞样”星形细胞组成。免疫组化显示肿瘤细胞多表达GFAP、Vim、NSE和S-100蛋白，不表达NFSyn；有1例表达EMA。随访5-27个月均生存。结论：SEGA为生长缓慢的良性肿瘤，生存时间长，预后良好，可能具有多向分化潜能，病理确诊须与巨细胞性胶质母细胞瘤、肥胖细胞性星形细胞瘤和室管膜瘤等鉴别。

OBJECTIVE： To study the clinicopathologicae, immunohistochemical characteristics and prognosis of subependyreal giant-cell astrocytoma（SEGA）. METHODS： Five cases of SEGA were studied by immunohistochemistry and cheanalysis of clinical characteristics, and all cases were followed up. RESULTS： The 5 cases, typically occurred during the first two decades of life. The predominant symptoms were headache and vomiting. All cases of SEGA were located in the wall of the lateral ventricles and associated with tuberous sclerosis. The histopathological features was the composition of large ganglioid astrocytes. Immunohistochemistry showed that the tumor cells were positive for GFAP, Vimentin, NSE and S-100 and negative for NF and Syn; and EMA was expressed in one case. The 5 cases living during the follow-up period of 5-27 months. CONCLUSION： SEGA is a benign, slowly growing and possible rnultidifferentiated tumor, and it should be differentiated from giant cell glioblastoma, gemistocytic astrocytoma and ependymoma.