Alveolar soft part sarcoma (ASPS) is a malignant soft tissue tumor that has an unclear origin. The term sarcoma assign to malignant tumors of tissues that connect, support, or surround other structures and organs of the body such as bone, cartilage, fat, muscle.
The term soft tissue indicates that ASPS is not a bone cancer, but is related to cancers that includes muscles, tendons, fibrous tissues, fat, blood vessels, nerves, and synovial tissues. The name Alveolar soft part sarcoma is derived from the histopathology of this cancer (microscopic pattern). ASPS was first described and characterized in 1952 by a pathologist named Christopherson. It is an extremely rare cancer. The exact cause of soft tissue sarcomas is not entirely understood, however, studies have indicated that genetic alterations may play a role.
ASPS is a slow growing tumor and is highly vascular. ASPS is a sarcoma, and that indicates that this cancer initially arises in soft tissues that connect, support, or surround other structures and organs of the body, rather than bones. Most alveolar soft part sarcoma cases occur in patients during their second and third decade of life with a slight female predilection. Most commonly, it involves the muscles and deep soft tissue of the pelvis and the extremities. Alveolar soft part sarcoma may exist in the patient’s body for a long time before being diagnosed. It can grow large and push aside surrounding tissues for a long time before causing any discomfort. Because alveolar soft part sarcoma affects tissue that is elastic and easily moved, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue.
The most common organs where ASPS metastasize into them are lungs and brain. Alveolar soft part sarcoma can enlarge to other areas of the body. It tends to recur or metastasize to the lung and brain years after diagnosis. Alveolar soft part sarcoma may be localized, meaning it has not spread beyond the tissue where it arose or beyond nearby tissues, or metastatic, meaning it has spread to lungs, brain or other parts of the body. Alveolar soft part sarcoma tumor may exist for a long time before being diagnosed. It can grow large and push aside surrounding tissues long before causing discomfort. Therefore ASPS symptoms may either be a painless swelling or a soreness caused by compressed nerves or muscles affecting the range of motion in the affected area. The metastatic tumors are similar to those of the primary ASPS tumor.
Surgery is the anchor of treatment for most soft tissue sarcomas, particularly for localized alveolar soft part sarcoma, which tends to be slow growing and unresponsive to chemotherapy. Current treatment results in good local control of primary tumors, but poor control of metastatic tumors. New approaches to treatment of metastatic alveolar soft part sarcoma must be investigated and applied. Surgery includes biopsy of surgical removal of the entire tumor. If surgery to remove the entire tumor isn't possible or the tumor has metastasized, surgery may be combined with radiation therapy, which uses high energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.
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