Desmoplastic (des-moe-PLAS-tik) small round cell tumors are a rare type of soft tissue cancer that typically begins in the abdomen.

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.

  • Teamwork. At Mayo Clinic, general surgeons, oncologists, radiation oncologists, radiologists and pathologists work as a multidisciplinary team to care for people with desmoplastic tumors. Other professionals are included as needed.
  • Experience. Though desmoplastic small round cell tumors are rare, Mayo Clinic doctors have extensive experience diagnosing and treating these cancers.
  • A full range of treatment options to consider. Mayo Clinic doctors will work with you to review all of your treatment options and choose the treatment that best suits your needs and goals. The range of treatments offered to people with desmoplastic tumors includes surgery, chemotherapy, radiation therapy and stem cell transplant.
  • Comprehensive cancer center. Mayo Clinic Cancer Center meets strict standards for a National Cancer Institute comprehensive cancer center, which recognizes scientific excellence and a multidisciplinary approach to cancer prevention, diagnosis and treatment.

Mayo Clinic in Rochester, Minn., and Mayo Clinic in Scottsdale, Ariz., are ranked among the Best Hospitals for cancer by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., is ranked high performing for cancer by U.S. News & World Report.

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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).

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

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. Chemtoherapy 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, to kill cancer cells. Radiation therapy may be recommended after surgery to kill any cancer cells that remain.
  • Stem cell transplant. In certain situations, your doctor may recommend a stem cell transplant to replace your bone marrow with bone marrow stem cells from your own body (autologous stem cell transplant). This procedure is also called a bone marrow transplant. High-dose chemotherapy is given before the stem cell infusion.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

At Mayo Clinic in Arizona, specialists in general surgery, hematology and oncology, and radiation oncology form a multidisciplinary team to care for people with desmoplastic tumors. Other experts are included as needed.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Florida, specialists in general surgery, hematology and oncology, and radiation oncology form a multidisciplinary team to care for people with desmoplastic tumors. Other experts are included as needed.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Minnesota, specialists in gastroenterologic and general surgery, pediatric surgery, oncology, pediatric hematology and oncology, and radiation oncology form a multidisciplinary team to care for children and adults with desmoplastic tumors. Other experts are included as needed.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Mayo Clinic doctors and scientists are studying new ways to diagnose and treat desmoplastic tumors.

Cancer research is conducted in coordination with the Mayo Clinic Cancer Center. The Mayo Clinic Cancer Center receives funding from the National Cancer Institute and is designated as a comprehensive cancer center — recognition for an institution's scientific excellence and multidisciplinary resources focused on cancer prevention, diagnosis and treatment.

See a list of publications by Mayo Clinic authors on desmoplastic small round cell tumors on PubMed, a service of the National Library of Medicine.

May 27, 2014