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A Retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) with unique pathologic and immunohistochemical features, presented as a renal tumor

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Ozden Tulunay M.D.
Professor of Pathology
Department of Pathology
School of Medicine
Ankara University Physical Address

Yasar Beduk M.D.
Professor of Urology
Department of Urology
School of Medicine
Ankara University Physical Address

Aysegul Yalcinkaya M.D.
Resident of Pathology
Department of Pathology
School of Medicine
Ankara University Physical Address

Resul Karaboga M.D.
Resident in Urology
Department of Urology
School of Medicine
Ankara University Physical Address

Citation: O. Tulunay, Y. Beduk, A. Yalcinkaya & R. Karaboga : A Retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) with unique pathologic and immunohistochemical features, presented as a renal tumor. The Internet Journal of Pathology. 2009 Volume 8 Number 2

Keywords: Desmoplastic small round cell tumor | Ewing’s sarcoma | immunohistochemistry | primitive neuroectodermal tumor | renal tumor | retroperitoneal/omental tumor

 

Abstract

We present a unique case of retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) in a young adult. This highly aggressive neoplasm must be distinguished from other primitive tumors that require different therapy. The tumor presented as a large mass originating from the right kidney, an evolving as a perirenal mass invading the kidney. Multiple recurrences within the following four post-operative years demonstrated a tumor with a unique location and the coexpression of FL-1 and WT1. Immunohistochemistry of the tumor shows the known immunophenotypic overlap between EWS/pPNET and DSRCT. The capacity of simultaneous multidirectional phenotypical expression of the tumor may serve a support to the concept that EWS/pPNET and DSRCT form single group tumors. The patient died of disease four years post-operatively. The accurate distinction between these entities has clear prognostic and therapeutic implications.


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