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  • Alveolar Soft Part Sarcoma (ASPS)

    Alveolar soft part sarcoma (ASPS) is a malignant soft tissue tumor, which are tumors that start in the soft connective tissues of the body such as fat, muscles or nerves. ASPS is slow-growing and usually starts in the legs or arms, although it can also be found in the head and neck. It 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 or your child notice any symptoms.
    • The cause of alveolar soft part sarcoma is unknown, but may be connected to genes.
    • Alveolar soft part sarcoma is a serious condition requiring surgical removal of the tumor and, in some cases, radiation.
    • Once the tumor is removed, children need to ongoing survivorship follow-up care because the tumor can return.

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

    Children and teens with alveolar soft part sarcoma are treated at Dana-Farber/Boston Children's through our Bone and Soft Tissue Tumor Program. Our integrated pediatric oncology program offers the combined expertise of a leading cancer center and a world-renown children’s hospital.

    • We provide the full set of options that can be used to treat bone and soft tissue tumors, and our solid tumor treatment team can help you determine which option is best for your child.
    • Our pediatric oncologists have access to, and often lead, some of the most innovative clinical trials for soft tissue sarcomas.
    • Our surgeons have specialized expertise in complex surgeries, and our radiation oncologists have access to the newest radiation therapy techniques that may also be used in the care of a child with soft tissue sarcoma

    What are alveolar soft part sarcoma symptoms?

    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 alveolar soft part sarcoma 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 is alveolar soft part sarcoma diagnosed?

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

    • x-rays, which produce images of internal tissues, bones, and organs onto film
    • magnetic resonance imaging (MRI), which produces detailed images of the area where the tumor is located
    • computerized tomography scan (CT or CAT scan) to capture a detailed view of the body, in some cases
    • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is collected during surgery
    • bone scan to detect bone involvement
    • complete blood count (CBC), which measures size, number and maturity of different blood cells in a specific volume of blood
    • other blood tests, including blood chemistries

    After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

    What are alveolar soft part sarcoma treatment options?

    Alveolar soft part sarcoma (ASPS) treatment may include:

    Surgery

    Surgery is often a first step, allowing doctors to form a complete diagnosis of the tumor type and providing information on the stage of the disease. If your child’s leg or arm is affected, your child may receive:

    • limb-salvage surgery, to help preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor
    • rotationplasty, a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone and uses the ankle as a knee joint
    • amputation surgery, which in rare cases may be necessary (for example, if it involves the nerves and blood vessels)

    Radiation therapy

    Radiation therapy can help stop the growth of abnormal cells in specific areas of the body. Radiation therapy uses high-energy rays from a specialized machine to damage or kill abnormal cells. Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery.

    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. As a result, surgery involves very careful planning. 

    • In some cases, before you and your child's treatment team decide on surgery, a radiologist may perform a procedure called arterial embolization. This blocks the blood flow in the abnormal vessels that are involved with the tumor. This reduces the risk of bleeding during surgery.
    • To determine whether your child can undergo arterial embolization without complications, your doctor may use an angiogram, a special x-ray that helps doctors to visualize the involved blood vessels.

    Alveolar soft part sarcoma is typically unresponsive to chemotherapy.

    What is the long-term outlook for children with ASPS?

    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. Local recurrence after surgical removal is uncommon; however, tumors can return many years after treatment. Children and young adults with alveolar soft part sarcoma require ongoing cancer survivorship care to monitor for recurrence and possible late effects related to treatment.