by Keila Torres, MD, PhD and Raphael Pollock, MD, PhD
Also available in Chinese, French, Italian, Japanese and Spanish
Abstract
Alveolar soft part sarcoma (ASPS) is a rare, poor prognosis neoplasm of unknown histogenesis with a distinctive histology, specific molecular characteristics, and unique clinical behaviors. ASPS generally develop in younger patients. Unlike other soft tissue sarcomas, ASPS also metastasizes to the brain. While surgery can improve outcomes even in the setting of metastatic disease, traditional chemotherapeutic agents and ⁄or radiotherapy have failed to demonstrate significant survival advantages. This article provides an overview of the clinical manifestations, diagnosis, radiographic
features, and treatment of ASPS.
Conclusions
In summary, the descriptive term ‘alveolar soft part sarcoma’ refers to a rare soft tissue sarcoma histologic subtype that typically occurs in young patients. It is characterized by a distinctive histological appearance and a specific molecular genetic abnormality; the prognosis is poor. Identification of a specific ASPS chromosomal translocation not only has provided critical information about the pathogenesis of this disease but also has led to rational molecular targeted therapy evaluation. Most series reported in the literature suggest that ASPS chemosensitivity is modest, providing a compelling rationale underlying a major role for surgery in localized disease. The occurrence of distant metastases is quite common in ASPS; however, even the largest currently published ASPS clinical series10,11 does not define the optimal treatment for metastatic ASPS disease. On the basis of our experience,11 the benefit of routine systemic chemotherapy on patients with metastatic disease is uncertain.
However, current treatment recommendations are based on very limited clinical information. In the meantime, new molecular targeted therapies, such as antiangiogenic approaches and tyrosine kinase inhibitors, comprise the most promising new approaches for the treatment of ASPS, a devastating tumor which unfortunately seems to emerge in patients who otherwise would be just on the cusp of normal adult life.
Last revision and medical review: 12/2010
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