摘要观察分析腹、盆腔促纤维组织增生性小圆细胞瘤(DSRCT)的CT表现特征及其化疗疗效。方法 回顾性分析2006年11月至2009年12月本院诊治的经病理证实的3例腹、盆腔DSRCT患者的CT影像表现,采用鬼臼乙叉甙(VP-16)+异环磷酰胺(IFO)+顺铂(DDP)或IFO+表阿霉素(EPI)化疗方案进行化疗,21 d为1个周期,其中病例1化疗2个周期、病例2化疗6个周期、病例3化疗4个周期。根据化疗前后最近两次的CT影像诊断,应用实体瘤疗效评价标准(RECIST)评价疗效。结果 CT显示DSRCT较典型的表现为腹、盆腔单发巨大或多发不规则结节或软组织肿块,与网膜、系膜及腹膜关系密切,原发位置较难确定;肿块密度多不均匀,内常见低密度坏死区,并可见斑点或片状钙化,增强后肿块轻～中等不均匀强化;较早出现淋巴结转移,晚期较常见肝、肺转移;晚期当肿块较大时,可压迫或侵犯邻近输尿管或肠管,引起肾积水或肠梗阻。多疗程联合化疗后3例患者均病情部分缓解(PR)。结论 DSRCT具有相对特征性的CT影像表现,多疗程联合化疗可控制病情发展。

abstractsObjective To investigate and analyze the CT features and chemotherapy efficacy of desmoplastic small round cell tumors (DSRCTs) in abdomen and pelvis. Methods Between November 2006 and December 2009, the CT images of 3 patients with pathology-confirmed DSRCTs in abdomen or pelvis in our hospital were retrospectively analyzed. The protocol of chemotherapy was epiopodophyllotoxin (VP-16)+ isofosfamide(IFO)+ cisplatin(DDP) or IFO+epirubicin (EPI), with 21 days as one cycle. Two cycles of chemotherapy were given to case 1, 6 to case 2, and 4 to case 3. The efficacy of chemotherapy was then evaluated using the response evaluation criteria in solid tumors (RECIST) according to two immediately consecutive CT scans performed respectively before and after chemotherapy. Results The CT showed that DSRCTs were typically featured by single large or multiple irregular nodules or soft masses in abdomen and pelvis. These lesions were in close anatomic relation with the omentum, mesenterium or peritoneum, whereas the primary lesion appeared difficult to identify. The masses were mostly uneven in density, usually with lowdensity necrotic regions inside, and spotty or patchy calcification as well. After use of contrast agent, mild to moderate heterogeneous enhancement was found in the masses. Lymph node metastasis could be present in the early stage, while the liver and lung metastasis were commonly seen in the advanced stage. Larger latestage lesions may compress or invade the adjacent urinary tracts or intestines to cause hydronephrosis or intestinal obstruction. Partial remission (PR) was achieved in the three cases after several courses of combined chemotherapy. ConclusionsDSRCT may show typical features on CT images. Multi-course combined chemotherapy may help control the disease progression.