促纤维组织增生性小圆细胞肿瘤临床病理研究

Desmplastic small cell tumors:a clinicopathological study

目的探讨促纤维组织增生性小圆细胞肿瘤的临床病理特点及鉴别诊断。方法复习3例促纤维组织增生性小圆细胞肿瘤患者的临床资料,标本采用常规石蜡切片,HE染色和EnVision免疫组化二步法染色。结果3例患者中,男性2例,女性1例,年龄为24、19及31岁。肿瘤位于腹腔、疝囊与左颞部皮下。镜下瘤细胞呈小圆形,形成大小和形状不一的上皮样细胞巢,在其周围有大量增生致密的纤维结缔组织包绕。免疫表型瘤细胞CK、EMA、vimentin、desmin和NSE(+)。结论根据本瘤的基本组织学形态及偶尔萌出的分化表现,结合免疫表型,可将本瘤分为常见的经典型及少见的分化型,并可与其他小细胞肿瘤做鉴别诊断。

Objective To explore the clinicopathological features and the differential diagnosis of desmplastic small cell tumors （DSCT）. Methods The clinical materials of three cases were reviewed, and the tumors were studied by using of paraffin sections, HE staining, immunohistochemical analysis （EnVision method） and observed under microscope three patients, two were male and one was female. The age of them was 24, 19 and 31 years, respectively. The tumors situated in the abdominal cavity, hernial sac and hypodermis of the temple. Microscopically, the characteristics of the tumors were that the tumor cells showed small round epithelial nests which were different in size and shape, and around the nests; there was a large quantity of dense connective fibrous tissues. By immunohistochemical staining, the tumor cells revealed positive for CK, vimentin, desmin and NSE. Condusions According to basic morphologic features and clinical manifestations of the tumor combined with the immunohistochemical findings, we could subdivide DSCT into two types, typical type and undifferentiated type, and distinguish them from other small cell neoplasms.