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Sarcoma
Volume 2012, Article ID 380896, 8 pages
http://dx.doi.org/10.1155/2012/380896
Review Article

Classification, Molecular Characterization, and the Significance of Pten Alteration in Leiomyosarcoma

1Department of Pathology, University of Utah, Salt Lake City, UT 84112, USA
2Molecular Medicine Program, University of Utah, Salt Lake City, UT 84112, USA
3Sarcoma Services, Huntsman Cancer Institute, Department of Orthopaedics, University of Utah, Salt Lake City, UT 84112, USA
4Center for Children's Cancer Research, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112, USA

Received 16 August 2011; Accepted 30 November 2011

Academic Editor: Alberto Pappo

Copyright © 2012 Allie H. Grossmann et al. 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.

Abstract

Leiomyosarcoma is a malignant smooth muscle neoplasm with a complicated histopathologic classification scheme and marked differences in clinical behavior depending on the anatomic site of origin. Overlapping morphologic features of benign and borderline malignant smooth muscle neoplasms further complicate the diagnostic process. Likewise, deciphering the complex and heterogeneous patterns of genetic changes which occur in this cancer has been challenging. Preliminary studies suggest that reproducible molecular classification may be possible in the near future and new prognostic markers are emerging. Robust recapitulation of leiomyosarcoma in mice with conditional deletion of Pten in smooth muscle and the simultaneous discovery of a novel role for Pten in genomic stability provide a fresh perspective on the mechanism of leiomyosarcomagenesis and promise for therapeutic intervention.