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促结缔组织增生性小圆细胞肿瘤(desmoplastic small round cell tumor,DSRCT)是极其罕见的肿瘤,好发于青年男性的腹腔。组织病理学特征性的表现为大量致密增生纤维中埋没着团状、索状排列的小圆型肿瘤细胞。细胞遗传学上具有特征性的11及22号染色体易位,即(t11;22)(p13;q12)易位,产生EWS-WT1融合基因,可以应用逆转录酶聚合酶链反应来检测新鲜组织或福尔马林固定组织中这一融合基因并确定诊断。预后非常差,中位生存时间17～25个月。尽管应用了联合化疗、肿瘤细胞减灭术及全腹盆腔放疗,治疗效果仍不理想。多种治疗方案正处在试验当中,如更多药物的联合化疗、诱导治疗后的靶向治疗、术后的腹腔热灌注化疗及调强放疗、肝转移后通过肝动脉应用(90Y)钇微球体进行栓塞治疗等。
Desmoplastic small round cell tumor (DSRCT) is an extremely rare tumor that occurs in the abdominal cavity of young men. Histopathology is characterized by the performance of a large number of dense proliferation of fibers buried in the group, cord-like arrangement of small round tumor cells. The cytogenetically characterized translocations of chromosomes 11 and 22, the (t11; 22) (p13; q12) translocation, generate the EWS-WT1 fusion gene and reverse transcriptase polymerase chain reaction can be used to detect fresh tissue Or formalin-fixed tissue in this fusion gene and confirm the diagnosis. The prognosis is very poor, the median survival time of 17 to 25 months. Despite the application of combination chemotherapy, cytoreductive surgery and pelvic radiotherapy, the therapeutic effect is still not satisfactory. A variety of treatment options are in the trial, such as more drugs combined with chemotherapy, induction therapy after targeted therapy, postoperative intraperitoneal hyperthermic chemotherapy and intensity modulated radiotherapy, liver metastasis through the hepatic artery (90Y) yttrium micro Sphere embolization treatment.