促纤维组织增生小圆细胞肿瘤3例报道及文献复习  

Intraabdominal desmoplastic small round cell tumour: a report of three cases and review of the literature

作　　者：刘保安[1] 粟占三[2] 匡艺昭[2] 

机构地区：[1]中南大学湘雅医学院病理学系,长沙410078 [2]湖南省肿瘤医院病理科,长沙410006

出　　处：《诊断病理学杂志》2004年第2期75-77,i003,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨促纤维组织增生小圆细胞肿瘤(DSRCT)的病理诊断要点。方法详细介绍了3例DSRCT的临床特征和病理学所见,并复习了有关文献。结果3例临床均以腹痛、腹块就诊,镜下所见相似。肿瘤细胞较小,核分裂象多,复合表达上皮、神经和肌源性多种组织的免疫表型标记。结论该瘤为罕见、侵袭力强的高度恶性肿瘤,主要见于年轻男性,绝大多数发生在腹腔内。瘤细胞具有神经、间叶和上皮标记复合表达,预后很差。Objective To investigate pathological features for diagnosis of desmoplastic small round cell tumour (DSRCT). Meth-ods Three cases of DSRCT were presented. Its clinical features and typical pathological findings were described in details and the related literature was reviewed. Results All the three cases presented as abdominal pain and mass before diagnosis. Under micro-scope, the tumor cell was small in size, high mitotic figures, and positive for epithelial and neural markers. Conclusion This ag-gressive and rare neoplasm predominantly affects males and is almost exclusively intraabdominal in location.It is unique that neu-ral, mesenchymal and epithelial markers are co-expressed with extremely poor prognosis.

关 键 词：促纤维组织增生小圆细胞肿瘤 文献复习 病理诊断 免疫表型标记 

分 类 号：R735.5[医药卫生—肿瘤]