腹腔内促纤维增生性小圆细胞肿瘤4例

Intra-abdominal desmoplastic small round cell tumor:an analysis of 4 cases

目的:探讨腹腔内促纤维增生性小圆细胞瘤(intra-abdominal desmoplastic small round cell tumor,IDSRCT)的临床特点.方法:对我院于1996-06/2006-10收治的4例IDSRCT患者诊疗及预后进行回顾性分析.结果:本研究报道4例患者,均表现为腹水和/或腹部肿物收入院,剖腹探查术发现肿瘤腹腔内广泛种植,术后病理证实为IDSRCT.结论:腹腔内促纤维增生性小圆细胞瘤是一种罕见腹膜恶性肿瘤,诊断时患者已经出现广泛转移且预后不良.

AIM： To investigate the clinical features of intraabdominal desmoplastic small round cell tumor （IDSRCT）. METHODS： We retrospectively analyzed 4 cases of IDSRCT in our hospital from June 1996 to October 2006. RESULTS： All the 4 cases were admitted with symptoms of ascites and/or abdominal mass. The patients underwent exploratory laparotomy, revealing multiple peritoneal and mesenteric masses with an extensive seeding. The diagnosis was made by pathology after operation. CONCLUSION： IDSRCT is a very rare peritoneal malignant neoplasm, and it is a disease with high metastasis and poor prognosis.