Disease Information for Alveolar soft parts sarcoma

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Clinical Manifestations
Signs & Symptoms
Abdominal Mass
Mass in Buttock
Mass in Lower Extremity
Mass in Muscle/Extremity
Mass in Thigh
Muscle Mass
Thigh Muscle Mass
Diagnostic Test Results
X-RAY
Xray/Lung consolidation/Chest
Disease Mechanism & Classification
Class
CLASS/Soft Tissue Involvement
Pathophysiology
Pathophysiology/Chromosomal translocation
Pathophysiology/Alveolar consolidation
Pathophysiology/Non-infectious alveolar consolidation
Pathophysiology/Unbalanced Translocation Somatic Mutation
Process
PROCESS/Neoplastic (category)
PROCESS/Primary soft tissue tumor (ex)
PROCESS/Sarcoma (ex)
PROCESS/Somatic mutation/not hereditary
Treatment
Drug Therapy - Indication
RX/Interferon alpha
Surgical Procedures or Treatments
SX/Surgery
Other Treatments
TX/Radiotherapy.
Definition

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]---------------

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External Links Related to Alveolar soft parts sarcoma
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Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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