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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 investigate the clinicopathological features and differential diagnosis of granulosa cell astrocytic tumors (GCA). Methods The clinical data of 2 patients with GCA were reported. Histological observation, histochemical staining, immunohistochemical staining, IDH1 / 2 gene mutation and 1p / 19 Missing detection. Results Two cases of GCA were female (57 years old) and male (37 cases), respectively. The tumors were located in the right ventricle and right basal ganglia respectively. Microscope by the large round / oval tumor cells, cytoplasm rich with eosinophilic granulocytes, and can be seen short-sphered tumor cells and interstitial lymphocyte infiltration. One case with focal calcification, one case showed mitotic, vascular proliferation and necrosis. Cytosolic granules periodic acid Schiff (periodic acid Schiff, PAS) positive, reticuloendothelial tissue surrounded by non-woven fibers around. The tumor cells were diffusely expressed vimentin, GFAP, S-100 and ATRX, partly expressed Olig-2, EMA and p53, but not expressed NeuN, IDH1R132H. One of the tumor cells expressed CD68; Ki-67 index was 2% and 20 %. No mutation of IDH1 / 2 and deletion of 1p / 19q were detected in 2 cases. Conclusions GCA is a rare subtype of astrocytoma characterized by intracytoplasmic eosinophilic tumor cells. Because of its histological morphology similar to that of foamy tissue cells, GCA is associated with a number of non-neoplastic lesions (such as infarcts , Demyelination) phase confused. In addition, its molecular typing and prognosis need to be further explored.