腹腔内促纤维增生性小圆细胞肿瘤是好发于男性青少年的罕见的高度恶性肿瘤 ,有特征性的病理及免疫组化表现 ,基因学检查可发现染色体存在特征性的t( 1 1 ;2 2 ) (p1 3 ;q1 2 )易位及EWS和WT基因融合 ,但临床表现往往不典型 ,容易发生误会 ,本文着重讨论腹腔内促纤维增生性小圆细胞肿瘤的临床诊断 ,旨在协助提高腹腔内促纤维增生性小圆细胞肿瘤的临床诊断水平。 Intraperitoneal fibrosing fibrovascular small cell tumors are rare in men and adolescents with a rare high degree of malignancy, there are characteristic pathological and immunohistochemical findings, chromosomes can be found chromosomally characterized t (1; 2 2) (p1 3; q1 2) translocations and EWS and WT gene fusion, but the clinical manifestations are often atypical and prone to misunderstanding. This article focuses on the clinical diagnosis of intraperitoneal fibromic small cell tumors to help improve Intraperitoneal promoting fibroinflammatory small round cell tumor clinical diagnosis.