Tumor desmoplásico de células pequeñas y redondas. Diagnóstico y tratamiento
- Markinez Gordobil, Izaskun
- Ruiz, Inmaculada
- Jiménez, Raúl
- Villarreal, Eloisa
- Lizarazu, Aintzane
- Borda, Nerea
- Arteaga, Xabier
- Medrano, Miguel Ángel
- Guisasola, Esther
- Beguiristain, Adolfo
- Navascués, José María E.
ISSN: 0304-4858, 2173-2302
Year of publication: 2012
Volume: 109
Issue: 3
Pages: 101-103
Type: Article
More publications in: Gaceta médica de Bilbao: Revista oficial de la Academia de Ciencias Médicas de Bilbao. Información para profesionales sanitarios
Sustainable development goals
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%.