title: Epithelioid sarcoma and unclassified sarcoma with epithelioid features: clinicopathological variables, molecular markers, and a new experimental model.
abstract: BACKGROUND
  Epithelioid sarcoma (ES) and unclassified sarcoma with epithelioid features (USEF) are clinically and therapeutically unresolved. We compared ES and USEF patients' clinical behavior, treatment, outcome, and molecular marker expression. Furthermore, preclinical ES study models were developed to enable comprehensive benchside investigations.
  
  
  METHODS
  A database of ES and USEF patients (n = 116) treated since 1992 was created. A clinically annotated ES-USEF tissue microarray (TMA) was assayed for tumor-related markers. Newly established human and commercially available ES cell lines were characterized and tested in vivo.
  
  
  RESULTS
  ES and USEF patients presenting with localized disease exhibited 22% and 25% local recurrence rates, 35% and 19% nodal metastasis rates, and 41% and 53% distant metastasis rates (median follow-up, 54 months and 39 months, respectively). The 5- and 10-year disease-specific survival rates were 88% and 43% and 52% and 42% (ES and USEF, respectively). TMA immunohistochemistry identified integrase interactor (INI)-1 loss, cancer antigen 125, and p53 nuclear expression as significantly more common in ES than USEF cases. Both cell lines preserved ES morphological and biochemical characteristics in vitro and in vivo; loss of INI-1 was shown to occur in both lines.
  
  
  CONCLUSIONS
  Enhanced knowledge of ES and USEF clinical behavior, marker expression, and molecular determinants, extended via experimental models, will hopefully accelerate development of urgently needed effective targeted therapies for ES and USEF.
authors.affiliation: Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas 77054, USA.
authors.forename: Aniket
authors.initials: A
authors.lastname: Sakharpe
authors.forename: Guy
authors.initials: G
authors.lastname: Lahat
authors.forename: Taher
authors.initials: T
authors.lastname: Gulamhusein
authors.forename: Ping
authors.initials: P
authors.lastname: Liu
authors.forename: Svetlana
authors.initials: S
authors.lastname: Bolshakov
authors.forename: Theresa
authors.initials: T
authors.lastname: Nguyen
authors.forename: Pingyu
authors.initials: P
authors.lastname: Zhang
authors.forename: Roman
authors.initials: R
authors.lastname: Belousov
authors.forename: Eric
authors.initials: E
authors.lastname: Young
authors.forename: Xianbiao
authors.initials: X
authors.lastname: Xie
authors.forename: Priya
authors.initials: P
authors.lastname: Rao
authors.forename: Jason L
authors.initials: JL
authors.lastname: Hornick
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Lazar
authors.forename: Raphael E
authors.initials: RE
authors.lastname: Pollock
authors.forename: Dina
authors.initials: D
authors.lastname: Lev
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D018394:CA-125 Antigen / Q000032:analysis; D045744:Cell Line, Tumor; D002648:Child; D002868:Chromosomal Proteins, Non-Histone / Q000032:analysis; D004268:DNA-Binding Proteins / Q000032:analysis; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D016158:Genes, p53; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D000071796:SMARCB1 Protein; D012509:Sarcoma / Q000235:genetics / Q000401:mortality / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000473:pathology; D015996:Survival Rate; D014157:Transcription Factors / Q000032:analysis; D016896:Treatment Outcome; D016159:Tumor Suppressor Protein p53 / Q000096:biosynthesis
chemical_list: D014408:Biomarkers, Tumor; D018394:CA-125 Antigen; D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; D016159:Tumor Suppressor Protein p53