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Alveolar soft part sarcoma



Alveolar soft part sarcoma Causes


In a chromosomal analysis of ASPS, the joining and breaking of 2 chromosomes in tumor cells are shown. One “chromosome x” breaks and joins chromosome 17. When this happens, a fusion between 2 genes named TFE3 and ASPL occurs, resulting in the formation of a fusion protein not found in normal, healthy cells.


Alveolar soft part sarcoma Definition


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 and young adults. ASPS is a highly angiogenic disease, which involves an intensive production of new blood vessels connecting the tumor to the blood and 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 and lungs. ASPS is a sarcoma that affects soft tissues that connects and supports organs and structures of the body. It usually develops in the deep soft tissues and muscles of the leg or thighs, but could also appear in hands, head and neck. However, it could also spread and develop inside bones.


Alveolar soft part sarcoma Diagnosis


Alveolar soft part sarcoma usually exists in a patient's body for years before being diagnosed. The cancer could grow and 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 and nerves. These symptoms may affect the range of motion in the affected area.


Alveolar soft part sarcoma Historical Background


Alveolar soft part sarcoma is a very rare cancer. Although sarcomas are only 1% of adult patients newly diagnosed with cancers and 15% of all cancers in children, ASPS occurs in less than 1% of sarcomas.


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