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目的提高对角蛋白阳性的间质网状细胞肿瘤(CIRCT)的认识。方法对1例CIRCT患者的临床表现、病理形态、免疫组化及超微结构进行分析,并复习相关文献。结果 CIRCT是一种罕见肿瘤,以成年男性为主,好发于淋巴结。肿瘤细胞中等至较大,圆形、卵圆形或梭形,胞质丰富,核圆形或椭圆形;部分瘤细胞界限不清,胞质浅嗜酸呈“合体细胞样”。瘤细胞呈弥漫束状或旋涡状,部分巢团状排列。免疫组化示肿瘤细胞CK8、CK18和vimentin弥漫(+),SMA和desmin灶性(+),p53(+),Ki-67阳性率为43%。超微结构示细而长的细胞突,不成熟细胞连接和核周包绕成束的中间丝。原位杂交EBV(-)。结论 CIRCT是罕见的恶性肿瘤,其诊断有赖于病理形态、免疫组化及超微结构的综合分析。p53过表达、转移和复发提示愈后较差。
Objective To improve the understanding of keratin-positive stromal reticulocyte tumors (CIRCT). Methods One case of CIRCT patients with clinical manifestations, pathological features, immunohistochemistry and ultrastructure were analyzed, and reviewed the relevant literature. Results CIRCT is a rare tumor, mainly in adult males, occurs in the lymph nodes. Tumor cells medium to larger, round, oval or fusiform, rich in cytoplasm, nuclear round or oval; part of the tumor cell boundaries unclear, cytoplasmic shallow eosinophilic “syncytial”. Tumor cells were diffuse bundle or whirlpool, part of the nest dangling. The positive rates of CK8, CK18 and vimentin in diffuse (+), SMA and desmin (+), p53 (+) and Ki-67 were 43% in immunohistochemistry. The ultrastructure shows thin and long cell processes, immature cell connections and perinuclear bundles of intermediate filaments. In situ hybridization EBV (-). Conclusion CIRCT is a rare malignant tumor, its diagnosis depends on the pathological morphology, immunohistochemistry and ultrastructure of a comprehensive analysis. p53 overexpression, metastasis and recurrence suggest that the worse after.