CAJ | 학술논문

　　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t（11；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。
　　촉결체조직증생성소원세포종류（desmoplastic small round cell tumor，DSRCT）시겁기한견적종류，호발우청년남성적복강。조직병이학특정성적표현위대량치밀증생섬유중매몰착단상、색상배렬적소원형종류세포。세포유전학상구유특정성적11급22호염색체역위，즉t（11；22）（p13；q12）역위，산생EWS-WT1융합기인，가이응용역전록매취합매련반응래검측신선조직혹복이마림고정조직중저일융합기인병학정진단。예후비상차，중위생존시간17～25개월。진관응용료연합화료、종류세포감멸술급전복분강방료，치료효과잉불이상。다충치료방안정처재시험당중，여경다약물적연합화료、유도치료후적파향치료、술후적복강열관주화료급조강방료、간전이후통과간동맥응용（90Y）을미구체진행전새치료등。
Desmoplastic small round cell tumors (DSRCTs) are a rare malignancy found in male adolescents that initially occur mostly in the abdominal cavity. Diagnosis is based on the histologic analysis of biopsies, which typically show small round blue cells in nests separated by abundant desmoplastic stroma. DSRCTs are associated with a unique chromosomal translocation t (11:22) (p 13;q 12) that involves the Ewing's sarcoma (EWS) gene and the Wilms' tumor (WT1) gene. Reverse transcriptase-polymerase chain reaction can be used to detect the fusion gene in fresh or paraffin-embedded tissues, which confirms the diagnosis. The prognosis is particularly poor. The median survival ranges from 17 to 25 months. Management of DSRCT remains challenging despite the use of aggressive ther-apies such as polychemotherapy, debulking surgery, and whole abdominal radiation. Several methods for improving patient survival are being evaluated, such as the addition of chemotherapy and targeted therapies to normal neoadjuvant protocols, complete surgical resec-tion with hyperthermic intraperitoneal chemotherapy, postoperative intensity-modulated radiation therapy, and yttrium-90 microsphere liver embolization for treating hepatic metastases.