Localized and metastatic myxoid/round cell liposarcoma

Clinical and molecular observations

Authors

  • Aviad Hoffman MD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Markus P. H. Ghadimi MD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
    3. Department of General, Visceral, and Pediatric Surgery, Heinrich Heine University, Duesseldorf, Germany
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  • Elizabeth G. Demicco MD, PhD,

    1. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Chad J. Creighton PhD,

    1. Division of Biostatistics, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
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  • Keila Torres MD, PhD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Chiara Colombo MD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Tingsheng Peng MD, PhD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Kristelle Lusby MD,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Davis Ingram BS,

    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Jason L. Hornick MD, PhD,

    1. Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
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  • Wei-Lie Wang MD,

    1. Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Vinod Ravi MD,

    1. Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Alexander J. Lazar MD, PhD,

    1. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Dina Lev MD,

    1. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. Department of Cancer Biology, The University of Texas MD Anderson Cancer Center, Houston, Texas
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  • Raphael E. Pollock MD, PhD

    Corresponding author
    1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas
    2. The Sarcoma Research Center, The University of Texas MD Anderson Cancer Center, Houston, Texas
    • Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 444, Houston TX 77030; Fax: (713) 563-4637

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  • The first 4 authors contributed equally to this article.

  • We thank the Lobo, Margolis, and Jackson Families for their continued support of our liposarcoma research and Dr. Torsten O. Nielsen for his helpful comments.

Abstract

BACKGROUND.

Myxoid liposarcoma (MLPS), a disease especially of young adults with potential for local recurrence and metastasis, currently lacks solid prognostic factors and therapeutic targets. The authors of this report evaluated the natural history and outcome of patients with MLPS and commonly deregulated protein biomarkers.

METHODS.

Medical records were retrospectively reviewed for patients who presented to the authors' institution with localized (n = 207) or metastatic (n = 61) MLPS (1990 to 2010). A tissue microarray of MLPS patient specimens (n = 169) was constructed for immunohistochemical analysis of molecular markers.

RESULTS.

The 5-year and 10-year disease-specific survival rates among patients with localized disease were 93% and 87%, respectively; male gender, age >45 years, and recurrent tumor predicted poor outcome. The local recurrence rate was 7.4%, and the risk of local recurrence was associated with recurrent tumors and nonextremity disease location. Male gender was the main risk factor for metastatic disease, which occurred in 13% of patients. Forty percent of patients who had localized disease received chemotherapy, mostly in the neoadjuvant setting. Immunohistochemical analysis revealed significantly higher expression of C-X-C chemokine receptor type 4 (CXCR4) and platelet-derived growth factor beta (PDGFR-β) in metastatic lesions versus localized lesions. Tumors with a round cell phenotype expressed increased levels of CXCR4, p53, adipophilin, PDGFR-α, PDGFR-β, and vascular endothelial growth factor relative to myxoid phenotype. Only the receptor tyrosine kinase encoded by the AXL gene (AXL) was identified as a prognosticator of disease-specific survival in univariate analysis.

CONCLUSIONS.

In this study, the authors identified clinical and molecular outcome prognosticators for patients with MLPS as well as several potential therapeutic targets. Cancer 2013. © 2013 American Cancer Society.

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