Desmoplastic small round cell tumors (DSRCTs) are extremely rare and mainly affect adolescents and young adults. The tumors are usually involved with the abdominal area and/or the pelvic peritoneum. Only a small number of cases have been reported concerning DSRCTs of the testicular region. The present study reports a case of DSRCT of the testis with radical orchectomy and systemic chemotherapy, leaving the patient disease-free for 14 months. However, the patient died of multiple metastasis 12 months later. Furthermore there is a review of the English literature to analyze the incidence, site of origin, imaging and pathological characteristics of DSRCT.
Keywords: desmoplastic small round cell tumor; testis.