Alveolar Soft Part Sarcoma Treatment

Alveolar soft-part sarcoma (ASPS) is a rare tumor that develops in soft tissues such as fat, muscle, and nerves. It occurs mainly in children but can also appear in adults. This type of sarcoma grows slowly but spreads and returns easily. It generally starts in the legs and arms, as well as the head and neck.

Alveolar soft part sarcoma usually appears as a non-painful lump in a leg or buttock. It does not usually produce symptoms right away. So, it can spread and grow before its cells have become large enough to be noticed and treated.
 

Alveolar soft part sarcoma symptoms

Alveolar soft part sarcoma can go unnoticed for a long time before it is diagnosed. When the tumor grows large enough, it starts pushing on the tissues and structures around it. Symptoms vary depending on the size and location of the tumor.

Signs and symptoms may include:

• A painless swelling or mass in the leg, buttocks, neck, or chest area
• Decreased range of motion in the legs or arms
• Limping
• Muscle stiffness
• Other symptoms caused by the tumor pressing on nerves or muscles in the affected.
   

Alveolar soft part sarcoma causes

The cause of alveolar soft part sarcoma is not clear. It is not passed on from parents to children through the genes.

Alveolar soft part sarcoma treatment

A team of doctors from many specialties will plan your child’s treatment. The standard of care is checkpoint inhibitors and immunotherapy. Checkpoint inhibitors are drugs that help keep an immune response from getting out of control and harming healthy cells.

Treatment may include:

• Surgery: A surgical oncologist (a cancer surgeon) removes the tumor and any surrounding tissues. This cancer often comes back after treatment. So, the surgeon may remove surrounding tissue to get all the cancer. If the tumor is in an arm or leg, this surgery might include removal of all or part of the arm or leg.
• Radiation therapy: A radiation oncologist uses light energy such as high-energy x-rays to destroy or reduce the size of tumor cells.
• Targeted drug therapy: Traditional chemotherapy medicines do not work well for this cancer. Targeted therapies that destroy the cancer cells without harming nearby healthy tissues are being studied in clinical trials.

Alveolar soft-part sarcoma prognosis

Alveolar soft part sarcoma is rare. Sarcomas make up about 15% of all childhood cancers. Only 1% of all sarcoma cases are alveolar soft part sarcoma. Fewer than 80 cases are diagnosed per year in the United States.

The prognosis for alveolar soft part sarcoma is usually poor. This is likely due to the lack of symptoms and the tendency to spread (metastasize) to other parts of the body. But the disease may progress slowly, and patients may live for years or decades after diagnosis. Children generally survive longer with the disease than adults do.

Alveolar soft part sarcoma care at St. Jude

• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
• St. Jude offers a dedicated team of specialists to meet the needs of children with cancer, including surgeons; doctors and nurses who treat this cancer; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosis (pathologists); experts who help manage and minimize the long-term or late effects of treatment; researchers; scientists; and many others.
• St. Jude has been a pioneer in limb-sparing surgical techniques. Our surgeons continue to explore new limb-sparing techniques to improve survival and help children live normal lives after treatment.
• The medical team works closely with laboratory researchers to bring new treatments from the lab to the clinic.

Alveolar soft part sarcoma clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults for alveolar soft part sarcoma. Learn more about clinical research at St. Jude.