颗粒细胞星形细胞肿瘤2例临床病理观察  

Granular cell astrocytoma:a report of two cases and review of literature

作　　者：洪柳[1] 孟宇宏[1] 宁浩勇[1] 吴惠[1] 周阳[1] 高逸群[1] 张冰[1] 段敏刚[1] HONG Liu MENG YU-hong NING Hao-yong WU Hui ZHOU Yang GAO Yi-qun ZHANG Bing DUAN Min-gang(Department of Pathology, Navy General Hospital of PLA, Beijing 100048, China)

机构地区：[1]海军总医院病理科,北京100048

出　　处：《诊断病理学杂志》2017年第6期435-439,444,共6页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨颗粒细胞星形细胞肿瘤(GCA)的临床病理特点及鉴别诊断。方法报道2例GCA患者的临床资料,并对其进行组织学观察及组织化学、免疫组化标记、异柠檬酸脱氢酶1/2(isocitrate dehydrogenase 1/2,IDH1/2)基因突变和1p/19缺失检测。结果 2例GCA分别为女性1例(57岁)、男性1例(37岁);肿瘤分别位于右侧脑室旁和右侧基底节。镜下由大的圆形/卵圆形肿瘤细胞构成,胞质丰富伴嗜酸性颗粒,并可见短梭形瘤细胞及间质淋巴细胞浸润。1例伴灶状钙化,1例可见核分裂、血管增生及坏死。胞质内颗粒状物过碘酸-希夫染色(periodic acid Schiff,PAS)阳性,网织染色示肿瘤细胞周围无网织纤维包绕。肿瘤细胞均弥漫表达vimentin、GFAP、S-100和ATRX,部分表达Olig-2、EMA和p53,不表达NeuN、IDH1R132H;其中1例部分肿瘤细胞表达CD68;Ki-67指数分别为2%和20%。2例均未检测到IDH1/2基因突变和1p/19q缺失。结论 GCA为一种罕见的星形细胞瘤亚型,以含有胞质内嗜酸性颗粒的肿瘤细胞为特征;由于其组织学形态与泡沫样组织细胞相似,易与一些非肿瘤性病变(如梗死、脱髓鞘)相混淆。此外,其分子分型特点和预后有待进一步探讨。Objective To explore the elinicopathologic characteristics and differential diagnosis of granular cell astrocytoma （GCA）. Methods The clinical data of two cases of GCA were reported. The histopathologic, histochemical, immunohistochemical features, isocitrate dehydrogenase 1/2 （IDH1/2） mutation and l p/19q delation were examined. Results Two GCA occurred in a 57-year-old woman and a 37-year-old man, and the tumors were located in the right periventricular and fight basal ganglia, respectively. Microscopically, the tumor was almost entirely composed of large cells with round to oval, granular eosinophilic cytoplasm, which were admixed with short spindle neoplastic cells and lymphocytic infiltration. In one case, a little calcification was observed. In another case, the tumor cells displayed karyokinesis, microvascular proliferation and necrosis. The large tumor cells with granular cytoplasm showing positive staining for periodicacid-Schiff （ PAS ） , and reticulin network were absent around the tumor cells. Immunohistoehemical analysis showed that tumor cells were diffusely positive for vimentin, GFAP, S-100 and ct-thalassemia/mental retardation syndrome X-linked （ ATRX）, and partially positive for oligodendrocyte transcription factor 2 （ olig 2）, epithelial membrane antigen （EMA） and p53, while negative for NeuN and IDH1R132H. In one case, CD68 was partially positive in granular tumor cells. Ki-67 labeling index was 2% and 20%, respectively. IDH1/2 mutation and lp/19q deletion were not detected in 2 cases. Conclusion GCA is a rare morphologic variant of astrocytomas, characterized by a prominent component of granular eosinophilic cytoplasm. As its histologic feature resembles that of a foamy histiocyte, GCA may be confused with some non-neoplastic diseases, such as infarct or demyelinating disease. In addition, its molecular type and prognosis need to be further explored.

关 键 词：脑肿瘤 颗粒细胞星形细胞肿瘤 组织化学染色 免疫组化 鉴别诊断 

分 类 号：R739.41[医药卫生—肿瘤]