Pleomorphic liposarcoma: Clinical observations and molecular variables

Markus P. Ghadimi, Ping Liu, Tingsheng Peng, Svetlana Bolshakov, Eric D. Young, Keila E. Torres, Chiara Colombo, Aviad Hoffman, Dominique Broccoli, Jason L. Hornick, Alexander J. Lazar, Peter Pisters, Raphael E. Pollock, Dina Lev

Research output: Contribution to journalArticlepeer-review

Abstract

BACKGROUND: Pleomorphic liposarcoma (PLS) is a rare high-grade sarcoma that has lipoblastic differentiation. In this study, the authors evaluated PLS natural history, patient outcomes, and commonly deregulated protein biomarkers. METHODS: Medical records from patients (n = 155) who had PLS from 1993 to 2010 were reviewed. Univariate and multivariate analyses were conducted to identify independent prognosticators. A PLS tissue microarray (TMA) (n = 56 patient specimens) was constructed for immunohistochemical analysis of molecular markers, and p53 gene sequencing (exons 5-9) was conducted. RESULTS: The average patient age was 57 years, and the patients presented with primary disease (n = 102), recurrent disease (n = 16), and metastatic disease (n = 37). Lower extremity was the most common disease site (40%), and the average tumor size was 11 cm. Complete follow-up data were available for 83 patients, and their median follow-up was 22.6 months. The 5-year disease-specific survival rate was 53%; and recurrent disease, unresectability, and microscopic positive margins were identified as predictors of a poor prognosis. Systemic relapse (the strongest poor prognostic determinant) developed in 35% of patients with localized PLS. Immunohistochemical analysis revealed increased expression of peroxisome proliferator-activated receptor gamma (an adipogenic marker), B-cell leukemia 2 and survivin (survival factors), vascular endothelial growth factor (an angiogenic factor), matrix metalloproteinase 2, and other biomarkers. Frequent loss of retinoblastoma protein expression and high p53 mutation rates (approximately 60%) were observed. CONCLUSIONS: PLS is an aggressive, metastasizing sarcoma. Identifying ubiquitous molecular events underlying PLS progression is crucial for progress in patient management and outcomes.

Original languageEnglish
Pages (from-to)5359-5369
Number of pages11
JournalCancer
Volume117
Issue number23
DOIs
Publication statusPublished - Dec 1 2011

Keywords

  • clinical outcome
  • molecular biomarkers
  • p53 mutations
  • pleomorphic liposarcoma
  • tissue microarray

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

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