Overview

Desmoplastic (des-moe-PLAS-tik) small round cell tumors are a type of soft tissue cancer that typically begins in the abdomen. Rarely, this type of cancer can occur in other parts of the body.

Desmoplastic small round cell tumors occur most often in young men and boys, though they can occur at any age and can also affect women.

These aggressive cancers often form as multiple tumors in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. They quickly spread to other structures within the abdomen.

Desmoplastic small round cell tumors are a type of soft tissue sarcoma.

Treatment for desmoplastic small round cell tumors typically involves a combination of treatments, such as surgery, chemotherapy and radiation therapy.

Desmoplastic small round cell tumors care at Mayo Clinic

April 25, 2017
References
  1. Goldblum JR, et al. Malignant soft tissue tumors of an uncertain type. In: Enzinger and Weiss's Soft Tissue Tumors. 6th ed. Philadelphia, Pa.: Saunders Elsevier; 2014. http://www.clinicalkey.com. Accessed Jan. 31, 2017.
  2. Honore C, et al. Abdominal desmoplastic small round cell tumor: Multimodal treatment combining chemotherapy, surgery and radiotherapy is the best option. Annals of Surgical Oncology. 2015;22:1073.
  3. Jordan AH, et al. Management of desmoplastic small round-cell tumors in children and young adults. Journal of Pediatric Hematology/Oncology. 2012;34(suppl):S73.

Desmoplastic small round cell tumors