Comparison of genetic, antigenic and clinical features of extra-osseous Ewing Sarcoma (EO-EWS) and o...
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Comparison of genetic, antigenic and clinical features of extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS). Amanda Rivera-Begeman; Carrye Cost; Stephen Lessnick; Richard Smith; Charles Timmons; Patrick Leavey Annual CTOS Meeting; Seattle, Washington, November 2007.

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Comparison of genetic, antigenic and clinical features of extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

Amanda Rivera-Begeman; Carrye Cost;

Stephen Lessnick; Richard Smith; Charles Timmons; Patrick Leavey

Annual CTOS Meeting; Seattle, Washington, November 2007


Background
Background extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • Ewing's sarcoma family of tumors (EFT)

    • Osseous EWS (O-EWS), Extraosseous ES (EO-EWS), Peripheral primitive neuroectodermal tumor (pPNET) and Askin's tumor of chest wall (Carvajal,R. et al; Hematol Oncol Clin North Am 2005)

  • Cell surface protein MIC-2 (CD99) expressed on both O-EWS and EO-EWS (Ambros, IM; Cancer; 1991)

  • FLI-1 nuclear immunostain + ve in 70% of EWS and PNET cases (Folpe et al; Am J Surg Pathol; 2000)


Background1
Background extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • EWS/FLI-1 is seen in patients with EFT (O’Sullivan,MJ et al; Hum Pathol; 2001)

  • Prior reports of treatment for EO-EWS demonstrated no advantage to the addition of Doxorubicin (Raney RB et al.; J Clin Oncol; 1997)

  • EO-EWS should be treated with strategies used for O-EWS vs. malignant mesenchymal tumors (Castex,M.P.; J Clin Oncol; 2007)


Objective
Objective extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • To describe genetic, antigenic, and clinical features of patients with EO-EWS, primarily those of intra-abdominal origin

  • To compare genetic and antigenic features of EO-EWS to those of randomly chosen patients with O-EWS


Patients
Patients extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • Eligibility criteria

    • EFT treated at Children’s Medical Center Dallas (1995 – 2005; n=52)

    • Availability of archival diagnostic material and clinical data


Patients1
Patients extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Methods
Methods extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • All immunostains were independently reviewed by 2 pathologists (CT, ARB)

  • Interpretation was subjective +ve vs. –ve

    • No grading was attempted


Fli-1 extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

weak positive

Fli-1

strong positive


CD99 (O13) extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

Weak positive

CD99 (O13)

strong positive


Results positive staining
Results – positive staining extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Results positive staining1
Results – positive staining extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Cytokeratin Cam 5.2 extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

positive

Carcinoembryonic antigen (CEA)

positive


Results positive staining2
Results – positive staining extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Results positive staining3
Results – positive staining extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Results positive staining4
Results – positive staining extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)


Methods1
Methods extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • RT-PCR

    • RNA extracted from formalin fixed, paraffin embedded tumor (Roche high pure RNA paraffin kit)

    • PCR was performed for EWS/FLI-1

      • EWS/FLI-1 fusion type identified by melt curve analysis

      • EWS-FLI-1 fusion type confirmed by agarose gel electrophoresis

    • Alternate partners were examined as necessary (ews/fev, etv1, etv4 and erg)


Results ews fli 1 type
Results – EWS-FLI-1 type extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • Not type 1 or 2

    • EO-EWS: confirmed t(11:22)(q24;q12) but no translocation products amplified

    • O-EWS: PCR product melting curve between types 1 and 2 but failed sequencing


Summary
Summary extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • Negative FLI-1 nuclear staining does not exclude EWS-FLI-1 translocation positive EFT

  • While CEA +ve staining can be seen in EO-EWS it does not differentiate this from O-EWS

  • More type 2 fusions noted in patients with EO-EWS


Conclusion
Conclusion extra-osseous Ewing Sarcoma (EO-EWS) and osseous Ewing sarcoma (O-EWS)

  • Variability may occur in immunostaining and genotype analysis of patients with extra-osseous Ewing sarcoma vs. osseous Ewing sarcoma.


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