Izaskun Markinez Gordobil, Inmaculada Ruiz, Raúl Jiménez, Eloisa Villarreal, Aintzane Lizarazu, Nerea Borda, Xabier Arteaga, Miguel Ángel Medrano, Esther Guisasola, Adolfo Beguiristain, José María E. Navascués
{"title":"Tumor desmoplásico de células pequeñas y redondas. Diagnóstico y tratamiento","authors":"Izaskun Markinez Gordobil, Inmaculada Ruiz, Raúl Jiménez, Eloisa Villarreal, Aintzane Lizarazu, Nerea Borda, Xabier Arteaga, Miguel Ángel Medrano, Esther Guisasola, Adolfo Beguiristain, José María E. Navascués","doi":"10.1016/j.gmb.2012.04.003","DOIUrl":null,"url":null,"abstract":"<div><p>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 <span>iv</span> 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%.</p></div>","PeriodicalId":35686,"journal":{"name":"Gaceta Medica de Bilbao","volume":"109 3","pages":"Pages 101-103"},"PeriodicalIF":0.0000,"publicationDate":"2012-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.gmb.2012.04.003","citationCount":"2","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Gaceta Medica de Bilbao","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0304485812000431","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 2
Abstract
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%.