Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumor (DSRCT) is an uncommon highly aggressive tumor that usually involves abdominal and pelvic peritoneum of adolescents and young adults, with a male predilection. It may present as a single mass, plaque-like areas, or as multiple nodules extending throughout the peritoneal cavity. Rare cases have been reported arising in the posterior cranial fossa, pleura, lung, bone, salivary glands, scrotum, ovary, and kidney.The tumor nodules are firm, yellowish-white, and frequently contain foci of hemorrhage, cystic change, and necrosis. Abdominal mass, pain, and ascites are the presenting symptoms. The medial survival is less than 3 yrs. after diagnosis. Extensive surgical debulking combined with chemotherapy, radiotherapy, and autologous bone marrow transplantation has improved survival in some cases. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission