The authors report a case of intraabdominal desmoplastic small round cell tumor in a 21-year-old male patient who presented with abdominal pain, right lower limb thrombosis and obstructive loss of renal function. Initial investigation showed a large lobulated intraabdominal soft tissue mass with internal cystic cavities, located posteriorly to the bladder and causing obstruction of ureteral flow, compression of the right iliac vein and partial occlusion of the rectum. Involvement of intraperitoneal and retroperitoneal nodes was also seen. Ultrasound, computed tomography and magnetic resonance imaging findings, as well as surgical and histopathology macroscopical, microscopical and immunohistochemistry findings are described.

Keywords: Desmoplastic small cell tumor, Desmoplastic tumor