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About Childhood Alveolar Soft Part Sarcoma (ASPS)

  • Alveolar soft part sarcoma (ASPS) is a malignant soft tissue tumor, a tumor that starts in the body's soft connective tissues such as fat, muscles, or nerves.

    ASPS is slow-growing and usually begins in the legs or arms. It can also form in the head and neck. ASPS can spread to other parts of the body and tends to come back years later.

    • Alveolar soft part sarcoma is very rare in children.
    • Because ASPS is slow-growing, it may be present for a while before you notice symptoms.
    • The cause of alveolar soft part sarcoma is unknown, but there may be a genetic connection.
    • Alveolar soft part sarcoma is a serious condition requiring surgical removal of the tumor and, in some cases, radiation therapy.
    • Once we remove the tumor, children need ongoing survivorship follow-up care because the tumor can return.

    At the Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our pediatric surgeons have specialized expertise in ASPS surgery. We treat children with ASPS in the Bone and Soft Tissue Treatment Program, where our solid tumor treatment team can help you determine which option is best for your child.

    Symptoms of Childhood Alveolar Soft Part Sarcoma

    ASPS may exist for a while before you detect any symptoms. Because the tumor affects elastic and easily moved soft tissues, it can grow large and push aside surrounding tissue without being noticed.

    Common ASPS 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

    How We Diagnose Childhood Alveolar Soft Part Sarcoma

    We use advanced imaging studies, bone scan, biopsy, and blood tests to diagnose ASPS.

    If we confirm a diagnosis, we will meet with you and your family to discuss the results and determine the best, most effective treatment options for your child.

    How We Treat Childhood Alveolar Soft Part Sarcoma

    Alveolar soft part sarcoma treatment typically combines surgery and radiation. It is generally unresponsive to chemotherapy.

    Currently, alveolar soft part sarcoma treatment is likely to include:

    • Surgery: Surgery is often the first step. It helps doctors form a complete diagnosis of the tumor type and provide information on the disease stage. If your child's leg or arm is affected, your child may need one of the following surgeries:
      • Limb-salvage surgery: The surgeon removes the tumor and healthy tissue surrounding the tumor while maintaining the function of the affected limb.
      • Rotationplasty: This is a partial amputation that preserves the cancer-free lower leg, attaches it to the thighbone, and uses the ankle as a knee joint.
      • Amputation: While it is only necessary in rare cases, a child may need amputation surgery if the tumor involves the nerves and blood vessels.
      • Arterial embolization: There is potential for blood loss during an operation to remove an alveolar soft part sarcoma because of the abnormal blood vessels that may be involved. In some cases, before you and your child's treatment team decide on surgery, a radiologist may perform a procedure called arterial embolization. It blocks the blood flow in the abnormal vessels involved with the tumor, reducing bleeding risk during surgery.
    • Radiation therapy: Radiation therapy uses high-energy rays from a specialized machine to damage or destroy abnormal cells. Our doctors use precisely targeted and dosed radiation to eliminate cancer cells left behind after your child's surgery.

    After treatment, we continue to provide long-term care to children through our pediatric cancer survivorship programs. We offer education and support as you and your family transition to survivorship and beyond.

    Long-term Outcomes for Children with Alveolar Soft Part Sarcoma

    The outlook for children and young adults with ASPS that has not spread is much better than for those whose disease has spread. In children without metastases, the five-year survival rate is 91 percent. It drops to around 60 percent if the disease has spread.

    Local recurrence after surgical removal is uncommon. However, tumors may return many years after treatment. Children and young adults with ASPS require ongoing cancer survivorship care to monitor recurrence and possible late effects related to treatment.

    Childhood Alveolar Soft Part Sarcoma Treatment Team

    The Dana-Farber/Boston Children's solid tumor team specializes in treating sarcomas in children. Our pediatric cancer experts offer the right diagnostic tests and the best treatments at the right time.

    View a complete list of the specialists in our Childhood Solid Tumor Center.