Desmoplastic small round cell tumors care at Mayo Clinic

Mayo Clinic specialists are respected for their expertise and experience caring for people with rare cancers, including those with desmoplastic small round cell tumors.

Diagnosis at Mayo Clinic

Tests and procedures used to diagnose desmoplastic small round cell tumors include:

  • Removing a sample of tissue for testing. A biopsy procedure involves removing samples of cells from the tumor. Doctors can collect tissue samples during surgery or with a needle that's passed through the skin.

    Tissue samples are sent to a laboratory for testing. Mayo Clinic's Molecular Anatomic Pathology Laboratory conducts technical and specialized testing, including molecular genetic tests. Using the latest technology, doctors in the lab are able to make an accurate and timely diagnosis.

  • Imaging tests. Imaging tests may be used to give your doctors a better indication of the extent of your cancer and whether it involves nearby structures.

    Imaging tests may include ultrasound, CT, MRI and positron emission tomography (PET).

Treatment at Mayo Clinic

Treatment options for desmoplastic small round cell tumors include:

  • Surgery. When possible, surgeons work to remove all of the cancer. It's not always possible though, if the tumor has grown to involve nearby structures. In these cases, your doctor may recommend other treatments to shrink the tumor first.

    When it's not possible to remove the cancer entirely, your surgeon may work to remove as much of the cancer as possible.

  • Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before surgery to shrink a tumor and make it more likely that it can be successfully removed during an operation. Chemotherapy may also be used after surgery to kill any cells that might remain after the operation.
  • Radiation therapy. Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill cancer cells. Radiation therapy may be recommended after surgery to kill any cancer cells that remain.
April 25, 2017
  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. 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.