Primary desmoplastic small round cell tumor of the duodenum

Abstract

The desmoplastic small round cell tumor (DSRCT) is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. A 14-year-old girl presented with intermittent epigastric pain; abdominal computed tomography and upper gastrointestinal barium X-ray revealed an 8 cm × 10 cm space-occupying mass in the duodenal region. The patient underwent pancreaticoduodenectomy and the final pathologic diagnosis was DSRCT. Although multi-agent systemic chemotherapy was given, the patient died of metastasis 8 months later. Early diagnosis and surgical treatment with adjuvant chemotherapy seems to be the best treatment choice for this disease.

Diagnosis of desmoplastic small‐round‐cell tumor by cytogenetic analysis: a case report

“…Complete surgical resection is also associated with improved survival. However, debulking surgery is usually impossible at an advanced stage . Despite such aggressive therapy, the outcome is poor.…”

Desmoplastic Small Round Cell Tumor

“…Neoplasms can also occur as primary intrascrotal (paratesticular) masses arising in continuity with the peritoneum via the processus vaginalis or in isolation, or as primary neoplasms within the thoracic cavity including the pleurae. 8 Much more rarely, DSRCT can arise as primary disease at other anatomic sites, including the head and neck (including the sinonasal cavity, salivary glands, and brain), [8][9][10] the hand, 8 and visceral organs such as small and large bowel, 11,12 ovary, 13 and kidney. 14,15 Tumors can present with widespread disseminated metastases including to the lungs and brain.…”