title: Pleomorphic liposarcoma: clinical observations and molecular variables.
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.
authors: Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors: Markus P
authors: MP
authors: Ghadimi
authors: Ping
authors: P
authors: Liu
authors: Tingsheng
authors: T
authors: Peng
authors: Svetlana
authors: S
authors: Bolshakov
authors: Eric D
authors: ED
authors: Young
authors: Keila E
authors: KE
authors: Torres
authors: Chiara
authors: C
authors: Colombo
authors: Aviad
authors: A
authors: Hoffman
authors: Dominique
authors: D
authors: Broccoli
authors: Jason L
authors: JL
authors: Hornick
authors: Alexander J
authors: AJ
authors: Lazar
authors: Peter
authors: P
authors: Pisters
authors: Raphael E
authors: RE
authors: Pollock
authors: Dina
authors: D
authors: Lev
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