Desmoplastic Small Round Cell Tumor: A Rare Case of Extraluminal Bowel Obstruction and Review of the Literature

Excerpt

Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignant neoplasm that was first described by Sesterhenn et al. [1]. Its estimated incidence is between 0.2 and 0.5 per million per year [2]. DSRCT has been characterized as a highly aggressive cancer, which predominantly affects male adolescents and young adults. It usually presents as multiple widespread peritoneal and omental implants within the abdomen and pelvis [3]. Peritoneal metastasis is almost always present at diagnosis, and concurrent extraperitoneal metastases have been found in 47% of patients [4]. Four good prognostic factors include absence of extraperitoneal metastasis, macroscopic resection of peritoneal disease, whole abdominopelvic radiotherapy, and postoperative chemotherapy. Herein, we describe a 25-year-old male patient who presented with constipation and abdominal distension and subsequently diagnosed with desmoplastic small round cell tumor. …