Desmoplastic (des-moe-PLAS-tik) small round cell tumors (DSRCT) are a type of cancer that often begins in the abdomen. Sometimes this type of cancer can occur in other parts of the body.
Desmoplastic small round cell tumors are rare cancers that begin as a growth of cells. The growths often form on the tissue that lines the inside of the abdomen and pelvis. This tissue lining is called the peritoneum. The cancer cells can quickly spread to other nearby organs. This might include the bladder, colon and liver.
Desmoplastic small round cell tumors can happen to anyone, but they're more common in young men and boys.
Treatment for desmoplastic small round cell tumors typically involves a combination of treatments. Options might include surgery, chemotherapy and radiation therapy.
Desmoplastic small round cell tumors are a type of soft tissue sarcoma. Soft tissue sarcoma is a term used to describe a large group of cancers that all start in the tissues that connect, support and surround other body structures.
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Desmoplastic small round cell tumor symptoms vary depending on where the cancer begins. Most often it begins in the abdomen.
Signs and symptoms of desmoplastic small round cell tumors in the abdomen include:
- Swelling of the abdomen
- Abdominal pain
- Difficulty urinating
When to see a doctor
Make an appointment with your health care provider if you have any persistent signs and symptoms that worry you.
It's not clear what causes desmoplastic small round cell tumors.
Doctors know that cancer begins when a cell develops changes in its DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply quickly. This creates a clump of cancer cells called a tumor. The cancer cells can invade and destroy healthy body tissue. In time, the cancer cells can break away and spread to other parts of the body.
Desmoplastic small round cell tumors care at Mayo Clinic
June 01, 2022
- Goldblum JR, et al. Other malignant soft tissue tumors, including those of uncertain type. In: Enzinger and Weiss's Soft Tissue Tumors. 7th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed April 29, 2022.
- Al-Ibraheemi A, et al. Desmoplastic small round cell tumors with atypical presentations: A report of 34 cases. International Journal of Surgical Pathology. 2019; doi:10.1177/10668969188171.
- Mora J, et al. Desmoplastic small round cell tumor 20 years after its discovery. Future Oncology. 2015; doi:10.2217/FON.15.32.
- Desmoplastic small round cell tumors (DSRCT). National Cancer Institute. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoplastic-small-round-cell-tumors. Accessed April 29, 2022.
- Subbiah V, et al. Multimodality treatment of desmoplastic small round cell tumor: Chemotherapy and complete cytoreducetive surgery improve patient survival. Clinical Cancer Research. 2018; doi:10.1158/1078-0432.CCR-18-0202.
- Chicago Consensus Working Group. The Chicago consensus of peritoneal surface malignancies: Management of desmoplastic small round cell tumor, breast and gastrointestinal stromal tumors. Cancer. 2020; doi:10.1002/cncr.32856.
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Desmoplastic small round cell tumors