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首页> 外文期刊>World Journal of Gastroenterology >Combined resection and multi-agent adjuvant chemotherapy for desmoplastic small round cell tumor arising in the abdominal cavity: report of a case.
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Combined resection and multi-agent adjuvant chemotherapy for desmoplastic small round cell tumor arising in the abdominal cavity: report of a case.

机译：联合切除联合多药辅助化疗治疗腹腔出现增生性小圆形细胞瘤：1例。

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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.
机译：增生性小圆形细胞瘤（DSRCT）是一种罕见的，高度侵袭性的恶性肿瘤，具有独特的组织学特征：密集的增生性基质中细胞生长的嵌套模式，发生于以男性为主的年轻人群中。平均生存期仅为约1.5-2.5年。在免疫组织化学研究中，肿瘤共表达上皮，肌肉和神经标志物。这项工作报告了一名出现呕血和慢性便秘的27岁男子。包括内窥镜检查，上消化道系列检查，腹部计算机断层扫描和钡灌肠研究在内的系列研究表明，内脏器官的分布受累。该患者接受了积极的手术，并接受了由5-氟尿嘧啶，环磷酰胺，依托泊苷，阿霉素和顺铂组成的术后辅助化疗。手术后他存活了20个月，没有任何疾病。尚未建立标准治疗方案。积极的手术结合术后多药辅助化疗是合理的，不仅可以缓解症状，而且可以尝试改善这一晚期DSRCT年轻患者的预后。

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