结缔组织增生性小圆细胞肿瘤2例并文献复习

DESMOPLASTIC SMALL ROUND CELL TUMOR:TWO CASES REPORT AND REVIEW OF LITERATURE

目的:分析结缔组织增生性小圆细胞肿瘤(DSRCT)的临床病理特征。方法:结合文献对2例相对少见的DSRCT进行临床、病理形态及免疫组化研究。结果:DSRCT由成团巢的小圆细胞和丰富的结缔组织间质组成。免疫组化:小圆形的肿瘤细胞vimentin(+);desmin(+);EMA(+);CK(+);NSE(+)。结论:DSRCT为高度侵袭性肿瘤,主要累及腹腔或盆腔的腹膜,肿瘤由小圆细胞和丰富的结缔组织间质组成。

Objective:To investigate the clinical and pathological features of desmoplastic small round cell tumor(DSRCT).Methods:We present two relatively rare cases of DSRCT focusing on its clinical,pathologic morphology and immunohistochemical features along with review of literature on this subject.Results:Histologic features revealed that the DSRCT was composed of nests or clusters of small round cells embedded in an abundant desmoplastic stroma.Tumor cells exhibited immunohistochemistry positive reac-tions for vimentin,desmin,epithelial membrane antigen(EMA),cytokeratins(CK)and neuron-specific enolase(NSE).Conclusion:DSRCT is a rare,highly aggressive neoplasm that mainly affects abdominal or pelvic cavity.Its main histologic feature is nests or clusters of small round cells embedded in an abundant desmo-plastic stroma.