UROLOGICAL ASPECTS OF INTRAABDOMINAL DESMOPLASTIC SMALL ROUND-CELL TUMOR OF CHILDHOOD - A PRELIMINARY-REPORT

To our knowledge intra-abdominal desmoplastic round cell tumor has not previously been described in the pediatric urological literature. Thi s lesion has only recently been recognized as a clinicopathological en tity with predilection for adolescent boys. We report on 2 patients wh o presented with urological symptoms and a large abdominal or pelvic t umor that was biopsied at exploratory laparotomy. Complete surgical ex cision was impossible in both patients, who subsequently underwent mul tiagent chemotherapy. Histological and immunohistochemical staining ar e distinctive in this condition. The tumor is associated with a poor p rognosis, despite multidisciplinary, multimodal therapy.