Desmoplastic small round cell tumor of ileum presenting as polyps: A rare presentation

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive, malignant tumor of undetermined histogenesis with a poor prognosis. Adolescent males are primarily affected with a typically abdominopelvic mass. Diagnosis is usually based on histologic analysis of biopsy and cytogenetic studies. Here, we report a 26-year-old adolescent male who presented with acute intestinal obstruction and was found to have multiple polypoidal masses in the small intestine. Histopathology showed small round cells arising from serosa infiltrating the submucosa and muscularis propria. Immunohistochemistry findings revealed positive staining for epithelial, mesenchymal, and neural markers, which confirmed the diagnosis of DSRCT. We report this rare presentation of DSRTC of the ileum with multiple polypoidal masses.