Tumor desmoplásico de células pequeñas y redondas. Diagnóstico y tratamiento

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%.