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Madisons Foundation - Moms And Dads In Search Of Needed Support
Alveolar Soft-Part Sarcoma (ASPS)
Tuesday, 15 October 2002
Last Updated Tuesday, 23 November 2004
What
Alveolar soft part sarcoma (ASPS) is a very rare, slow-growing tumor that arises in soft tissues. ASPS is malignant, meaning it can spread and invade other tissues. ASPS is only found in 1 percent of all sarcomas (cancerous growths of the soft tissue), and sarcomas themselves are only 1 percent of all cancers, so there isn’t a great deal of information available about it. There are fewer than 100 cases in the United States each year. This kind of tumor often arises in the thigh or leg, although it may be found at first in the head and neck. Tumors can be localized, staying within or near the soft connective and muscle tissues from which they grow, or they can metastasize, as cancer cells travel via the bloodstream to other parts of the body, such as the lung and brain.Who
ASPS is more common in girls than boys. Although it can occur at any age, most people who get ASPS are younger than age 40.Signs and Symptoms
Because ASPS affects tissue that is soft and elastic, a tumor may exist for a long time before it is large enough to be discovered. A child may experience symptoms in many different ways, including a painless swelling or lump, pain or soreness when nerves or muscles are compressed, limping or other problems using the legs and feet, and a lower than normal range of motion in the affected area. However, symptoms of ASPS can resemble other conditions or medical problems. You should always consult your child's physician for a diagnosis.Possible Causes
ASPS seems to be caused by a genetic error called an “unbalanced translocation,” which requires two chromosomes to break apart and recombine. This genetic alteration has been observed in essentially all ASPS examined so far. However, it cannot be passed on to your relatives, as the translocation occurs only in the tumor, not in your child’s normal cells. Some inherited (genetic) diseases also seem to increase the chance of developing soft tissue sarcomas.Diagnosis
To confirm the diagnosis of ASPS, your child’s doctor will want to take a biopsy. This is a simple surgical procedure in which a single tissue sample is taken from the tumor. Examination using a microscope will determine what kinds of cells are present and enable the doctor to identify exactly what kind of cancer may be present. If the diagnosis is ASPS, the doctor will determine what stage the tumor has reached, which will confirm whether or not the tumor has spread from its original location, and, if so, how far. “Staging” helps doctors decide what kind of treatment is best, and gives an idea as to what the prognosis will be. This is accomplished through noninvasive imaging studies that may be needed, including X-rays, Magnetic Resonance Imaging (MRI), a Computerized Tomography scan (CT or CAT scan) and bone scans. Blood tests probably will be ordered, as well.Treatment
The main way to treat ASPS is to remove the entire tumor by surgery, as well as a portion of the surrounding healthy tissue, to make sure there are no remaining cancer cells. If surgery to remove the whole tumor isn't possible, or the tumor has metastasized, radiation therapy may be needed. Chemotherapy may also be used if the tumor has metastasized, but otherwise, it is not as effective as surgery.Prognosis
Prognosis always depends on many factors, such as the extent of the disease, size and location of the tumor, the presence or absence of metastasis, the tumor's response to therapy, the age and overall health of your child, your child's ability to tolerate specific medications, procedures, or therapies, and new developments in treatment. Children with ASPS tend to do better than adults. However, continuous follow-up care will be essential, as the disease can occur again, even many years later.Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
The Alliance Against Alveolar Soft Part Sarcoma
A great website for parents to find links to support groups, ongoing research and clinical trials and other informational sites.
National Cancer Institute
A good website for general information, links to current research and support groups. There is also nice definitions for many technical terms.
The sarcoma Allliance
A general website with information on all types of sarcomas, including some specific to ASPS, that provides education, guidance and support for parents and families affected by the disease.