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Volume 2012, Article ID 428789, 9 pages
Review Article

Alveolar Soft Part Sarcomas: Molecular Pathogenesis and Implications for Novel Targeted Therapies

1Division of Pediatric Hematology/Oncology, Department of Pediatrics, Mattel Children's Hospital at UCLA, UCLA David Geffen School of Medicine, 10833 Le Conte Avenue, Los Angeles, CA 90095-175, USA
2UCLA Pediatric Bone and Soft Tissue Sarcoma Program, The UCLA Sarcoma Program, Nanotechnology Program Area, UCLA Jonsson Comprehensive Cancer Center, Los Angeles, CA 90095, USA

Received 2 December 2011; Accepted 29 January 2012

Academic Editor: C. Fisher

Copyright © 2012 Bryan Mitton and Noah Federman. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Alveolar soft part sarcoma (ASPS) is a very rare soft tissue sarcoma which arises primarily in children and young adults. Despite its unique histology and well-characterized genetic translocation, many questions remain regarding the pathogenesis and treatment of this tumor type. Though collective clinical experience with this tumor type spans more than 60 years, there has been little progress made in treating this uncommon but frequently fatal disease. This paper focuses on the available data regarding its molecular pathogenesis and insights into targeted therapeutics as well as the results of clinical trials performed to date to hopefully improve the outcome of patients with this rare malignancy.