ALVEOLAR SOFT PART SARCOMA: WHAT IS ALVEOLAR SOFT PART SARCOMA?
Definition: What is "Alveolar soft part sarcoma"?
Alveolar soft part sarcoma or "ASPS", which was first identified in 1952, is an extremely rare type of soft tissue sarcoma, a slow-growing tumor that occurs mainly in children & young adults. ASPS is a highly angiogenic disease, which involves an intensive production of new blood vessels connecting the tumor to the blood & enabling dissemination of tumor cells into the bloodstream. Because of this, tumor cells can easily transfer into various parts of the body, usually affecting the brain & lungs. ASPS is a sarcoma that affects soft tissues that connects & supports organs & structures of the body. It usually develops in the deep soft tissues & muscles of the leg or thighs, but could also appear in hands, head & neck. However, it could also spread & develop inside bones.
In a chromosomal analysis of ASPS, the joining & breaking of 2 chromosomes in tumor cells are shown. One "chromosome x" breaks & joins chromosome 17. When this happens, a fusion between 2 genes named TFE3 & ASPL occurs, resulting in the formation of a fusion protein not found in normal, healthy cells.
Alveolar soft part sarcoma usually exists in a patient's body for years before being diagnosed. The cancer could grow & even push aside its surrounding tissues before it causes any discomfort. As such, ASPS symptoms are usually painless soreness or swelling caused by compressed muscles & nerves. These symptoms may affect the range of motion in the affected area.
Alveolar soft part sarcoma is a very rare cancer. Although sarcomas are only 1% of adult patients newly diagnosed with cancers & 15% of all cancers in children, ASPS occurs in less than 1% of sarcomas.