• 作者：Izaskun Markinez Gordobil ; ^{ikur6@hotmail.com} ; Inmaculada Ruiz ; Raú ; l Jimé ; nez ; Eloisa Villarreal ; Aintzane Lizarazu ; Nerea Borda ; Xabier Arteaga ; Miguel Á ; ngel Medrano ; Esther Guisasola ; Adolfo Beguiristain ; José ; Marí ; a E. Navascué ; s
• 关键词：Tumor desmopl&aacute ; sico de c&eacute ; lulas peque&ntilde ; as y redondas ; Translocaci&oacute ; n ; EWS ; WT1
• 刊名：Gaceta M茅dica de Bilbao
• 出版年：2012
• 期刊代码：pca165_03044858
• 类别：med
• 出版时间：July-September, 2012
• 卷：109
• 期：3
• 页码：101-103
• 文件大小：334 K

Desmoplastic small-round-cell tumor is an uncommon highly aggressive type of cancer that affects young men. We present the case of a 31-year-old man with stage iv desmoplastic small-round-cell tumor, diagnosed in the context of abdominal pain and hemoperitoneum. Surgery confirmed extensive hemoperitoneum and revealed multiple peritoneal nodules. Hemostasis was performed, a biopsy was taken, and chemotherapy was initiated. Seven months after surgery, the patient is still alive. Desmoplastic small-round-cell tumor usually occurs in the abdominal or pelvic peritoneum and is associated with nonspecific signs and symptoms but with a unique cytogenetic profile [a translocation t(11;22)(p13;q12) affecting the EWS and WT1 genes]. The best outcomes are produced by a combination of chemotherapy, radiotherapy and surgery. Nevertheless, the prognosis of desmoplastic small-round-cell tumor is poor, with an overall 5-year survival rate of 15%.