• Alveolar Soft Part Sarcoma Overview

    Alveolar soft part sarcoma (ASPS) is a very rare cancer in adolescents and young adults that starts in connective tissues of the body, including fat, muscles and nerves. These slow-growing tumors usually arise in the legs or arms, but can also start in the head or neck. 

    ASPS may spread to other parts of the body and, in some cases, may be cured with a combination of surgery and radiation therapy. Unfortunately, tumors can often return after treatment, even many years later.

    Alveolar Soft Part Sarcoma (ASPS) Treatment at Dana-Farber/Boston Children's

    Children and young adults with ASPS are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Bone & Soft Tissue Program. Continue reading to learn more about alveolar soft part sarcoma or visit the Bone & Soft Tissue Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    Because these tumors affect soft tissues, which are elastic and easily moved, a tumor may exist for a long time before being discovered, growing very large and pushing aside surrounding tissue. The most common symptoms include:

    • Painless swelling or lump
    • Pain or soreness caused by compressed nerves or muscles
    • Limping or other difficulty using the legs and feet
    • Diminished range of motion in the affected area

    In addition to a complete physical examination, doctors diagnose ASPS with:

    • X-ray
    • Magnetic resonance imaging (MRI)
    • Computerized tomography (CT or CAT) scan
    • Biopsy
    • Bone scan to determine the cause of pain and inflammation
    • Complete blood count and other blood tests

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Alveolar soft part sarcoma is treated with:

    • Surgery – particularly for disease that has not spread. This may include limb-salvage surgery to remove the tumor and some healthy tissue around it or amputation if the tumor cannot be removed because it involves major nerves or blood vessels.
    • Radiation therapy – to shrink the tumor and control growth if it cannot be surgically removed
    • Arterial embolization – to block blood flow in abnormal vessels involved with the tumor and reduce the risk of bleeding during surgery

    The disease is typically unresponsive to chemotherapy.

    Progressive or Recurrent Disease

    Local recurrence after surgical removal is uncommon; however, tumors can return many years after treatment.

    Long-term Outlook

    The outlook for children and young adults with ASPS that has not spread is much better than it is for those whose disease has spread. In children without metastases, the five-year survival rate is 91 percent, but falls to around 60 percent if the disease has spread.  Children and young adults with alveolar soft part sarcoma require ongoing care to monitor for recurrence.
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