Disease Information for Alveolar soft parts sarcoma

Alveolar Soft Part Sarcoma; ASP sarcoma; ASPS; Alveolar sarcoma of soft parts; very rare cell-type sarcoma usually in lower limb or buttock muscle tissue but connective tissue cell type; Alveolar soft part sarcoma (ASPS) is a rare sarcoma (malignant tumor of connective tissue) of an unclear cause; It is among the least common sarcomas, representing 0_2-1 percent of large studies of soft tissue sarcomas; Since there are approximately 8000 soft-tissue sarcomas per year that arise in the United States, this means there are on the order of 15 to 80 cases nationwide each year; It is characterized by a painless mass in the leg or buttock, with a particular affinity to travel to the lungs as multiple nodules, presumably while the sarcoma itself is still small; ASPS is very rare, because it involves a specific breaking and joining event between two chromosomes, called an "unbalanced translocation"; This finding is observed in essentially all ASPS examined so far; This finding cannot be passed on to children, however, as the finding occurs only in the tumor, not in the normal cells; in addition, there are no families in which multiple family members have ASPS; Treatment is with surgery and radiation for the primary place where the sarcoma arises; For disease that travels to the lungs, sometimes surgery is possible to remove nodules, but more typically chemotherapy is the only option for treatment; This tumor tends to be resistant to standard chemotherapy, so new treatments involving new chemotherapy drugs are also a reasonable option for treatment; Interferon-alfa showed benefit in one person from Japan with ASPS; There is little other data on possible helpful chemotherapy for this tumor--------------NORD 2005]---------------

(Edit)