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ALVEOLAR SOFT PART SARCOMA: WHAT IS ALVEOLAR SOFT PART SARCOMA?

Read about alveolar soft part sarcoma medical facts: what is the definition of alveolar soft part sarcoma, information about the causes, diagnosis, historical background, and related alveolar soft part sarcoma diseases.

Definition: What is "Alveolar soft part sarcoma"?

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.

Causes

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.

Diagnosis

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.

Historical Background

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.

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