Epithelioid sarcoma and unclassified sarcoma with epithelioid features: Clinicopathological variables, molecular markers, and a new experimental model

Aniket Sakharpe, Guy Lahat, Taher Gulamhusein, Ping Liu, Svetlana Bolshakov,Theresa Nguyen, Pingyu Zhang, Roman Belousov, Eric Young, Xianbiao Xie, Priya Rao, Jason L. Hornick, Alexander J. Lazar, Raphael E. Pollock, Dina Lev

Research output: Contribution to journalArticlepeer-review

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. Patients and 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.

Original languageEnglish
Pages (from-to)512-522
Number of pages11
JournalOncologist
Volume16
Issue number4
DOIs
StatePublished - 2011
Externally publishedYes

Keywords

  • Animal model
  • CA-125
  • Cell lines
  • Epithelioid sarcoma
  • INI-1
  • Molecular markers

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