角蛋白阳性的间质网状细胞肿瘤临床病理观察

Clinicopathological faetures of cytokeratin-positive interstitial reticulum cell tumors

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 recognition of cytokeratin-positive interstitial reticulum cell tumor（CIRCT）.Methods The clinicopathological features of one case of CIRCT were examined by light and electron microscopy and immunohistochemical study,with review of the pertinent literature.Results As a rare malignant tumor,the patients were predominated in adult males with lymph node involvement.The neoplastic cells ranged from medium to large in size and from round to oval to spindle in shape,with elongated or round nuclei,prominent nucleoli and abundant cytoplasm.Some of them had a faintly eosinophilic cytoplasm with indistinct cell borders resulting in a ＂syncytical＂.These cells were arranged in a diffusely fascicular and vaguely whorled pattern,and some in nests.The tumor cells stained diffusely for cytokeratins 8 and 18,vimentin,and focally for SMA and desmin.Intense p53 expression and high cell proliferation index were related with aggressive clinical course with fatal outcome.Electron microscopy revealed slender and long cytoplasmic projections,rudimentary cell junctions,and perinuclear intermediate filament.In-situ hybridization for Epstein-Barr virus was negative.Conclusion CIRCT is a rare malignant tumor.The diagnosis of this tumor should be combined with pathomorphological features,immunohistochemical study,and ultrastructural observation.P53 over-expression,metastasis,and relapse suggest the poorer prognosis.