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.
Diagnosis
Desmoplastic small round cell tumors are diagnosed with a procedure to remove a sample of cells for testing. This cancer happens when abnormal cells form in the soft tissues. It usually happens in the tissue that lines the inside of the belly. To remove the cells, you might need surgery or a needle can be passed through the skin. The cells are sent to a lab for testing. Imaging tests might also be needed, like ultrasound, CT, MRI, and PET.
Treatment
Surgery is usually used to treat desmoplastic small round cell tumors. This cancer happens when abnormal cells form in the soft tissues. It usually happens in the tissue that lines the inside of the belly. Surgeons work to remove as much of the cancer as they can. Other treatment options might include chemotherapy with strong drugs and radiation with powerful energy beams.