摘要
Desmoplastic small round cell tumor （DSRCT） is a rare, highly aggressive malignancy with distinctive histological features： a nesting pattern of cellular growth within dense desmoplastic stroma, occurring in young population with male predominance. The mean survival period is only about 1.5-2.5 years. The tumor has co-expressed epithelial, muscle, and neural markers in immunohistochemical studies. This work reports a 27-year-old man presenting with hematemesis and chronic constipation. Serial studies including endoscopy, upper gastrointestinal series, abdominal computed tomography and barium enema study showed disseminated involvement of visceral organs. The patient underwent aggressive surgery and received postoperative adjuvant chemotherapy consisting of 5-fluorouracil, cyclophosphamide, etoposide, doxorubicin, and cisplatin. He survived without any disease for 20 mo after the surgery. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome in this advanced DSRCT young patient.
促结缔织增生的小圆房间肿瘤(DSRCT ) 是有特殊组织学的特征的稀罕、高度好攻击的恶意：在稠密的促结缔织增生基质以内的细胞的生长的一个嵌套的模式，与男优势发生在年轻人口。吝啬的幸存时期仅仅关于 1.5-2.5 年。肿瘤共同表示上皮，在免疫的肌肉，和神经标记组织化学的研究。这个工作报导与吐血和长期的便秘介绍的一个 27 岁的人。包括内视镜检查法，上面的胃肠的系列，腹的计算断层摄影术和钡灌肠的连续研究学习内脏的机关的显示出的传播参与。病人经历了好攻击的外科并且收到了由 5 氟尿嘧啶，出租机动三轮车磷酰胺， etoposide， doxorubicin，和 cisplatin 组成的手术后的辅助化疗。没有任何疾病，他在外科以后为 20 瞬间幸存。没有标准疗法协议被建立了。与手术后的多代理人助手化疗相结合的好攻击的外科被认为正当不仅减轻症状而且试着在这个先进 DSRCT 年轻病人改进结果。
