title: Localized and metastatic myxoid/round cell liposarcoma: clinical and molecular observations.
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.
authors: Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors: Aviad
authors: A
authors: Hoffman
authors: Markus P H
authors: MP
authors: Ghadimi
authors: Elizabeth G
authors: EG
authors: Demicco
authors: Chad J
authors: CJ
authors: Creighton
authors: Keila
authors: K
authors: Torres
authors: Chiara
authors: C
authors: Colombo
authors: Tingsheng
authors: T
authors: Peng
authors: Kristelle
authors: K
authors: Lusby
authors: Davis
authors: D
authors: Ingram
authors: Jason L
authors: JL
authors: Hornick
authors: Wei-Lie
authors: WL
authors: Wang
authors: Vinod
authors: V
authors: Ravi
authors: Alexander J
authors: AJ
authors: Lazar
authors: Dina
authors: D
authors: Lev
authors: Raphael E
authors: RE
authors: Pollock
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000704:Analysis of Variance; D014408:Biomarkers, Tumor / Q000032:analysis*; D017024:Chemotherapy, Adjuvant; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D008080:Liposarcoma / Q000737:chemistry* / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D018208:Liposarcoma, Myxoid / Q000737:chemistry / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000453:epidemiology; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors; D046888:Tissue Array Analysis; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor