Alveolar Soft Part Sarcoma

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Alveolar soft part sarcoma (ASPS) is a rare malignant neoplasm that is of unknown cell differentiation. Skeletal muscle differentiation has been proposed but is not strongly supported. ASPS is a slow-growing malignancy with a high propensity for metastasis; therefore early detection and treatment is important.

Clinically, it is more common in adolescent or young adults, with predilection for occurrence in young females. In at least two studies, females were found to be predominant (68-71%) among patients less than 30 years of age and males were predominant (67-68%) among patients over 30 years of age. It is more commonly described in the lower limb and around the limb girdle. These tumors are slow growing but have a very high predilection for metastasis, especially to the lungs, brain, bone, liver, spleen and other unexpected areas such as the female genital tract cervix and uterus. There is only one published report (from 2010) that describes metastasis to the mandible. This patient was known to have ASPS which metastasized to the mandible after one year of diagnosis. Most series have suggested that ASPS is resistant to conventional chemotherapy agents and that complete surgical excision is the treatment of choice for primary and metastatic lesions. Radiotherapy as a treatment for ASPS is still not well studied, but anti-angiogenic types of medications show a promising outcome. Survival seems to be associated with age and the size of the tumor at diagnosis; older patients and tumors larger than 10 cm have a poor prognosis. Larger tumor size appears to be associated with increased risk of distant metastases in adult patients. One study showed the median survival of 26 patients was 90 months, with overall 5- and 10-year survival rates of 64% and 48%, respectively, but the median survival for those with stage IV disease at initial diagnosis was 41 months. ASPS has a 30% incidence of brain metastases. These have a much poorer prognosis; patients typically survive one year after diagnosis.


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