Annals of Diagnostic Pathology
Volume 10, Issue 6 , Pages 363-366, December 2006

Ewing sarcoma/primitive neuroectodermal tumor of the kidney: a case report. Diagnosed by immunohistochemistry and molecular analysis

  • Rakhee Saxena, MD

      Affiliations

    • Department of Surgical Pathology and Laboratory Medicine, Buffalo General Hospital, Buffalo, NY 14263, USA
    • ,
  • Sheila Sait, PhD

      Affiliations

    • Department of Surgical Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY 14263, USA
    • ,
  • Paulette Mhawech-Fauceglia, MD

      Affiliations

    • Department of Surgical Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY 14263, USA
    • Corresponding Author InformationCorresponding author. Department of Pathology, Roswell Park Cancer Institute, Buffalo, NY 14263, USA. Tel.: +1 716 845 3204; fax: +1 716 845 3427.

Abstract 

Background

Ewing sarcoma/primitive neuroectodermal tumor (EWS/PNET) of the kidney is a rare and aggressive tumor. It has a rapid clinical progression with early metastasis and death. Few cases with documented t(11;22) have been reported in the literature.

Case Presentation

We report a case of EWS/PNET of the kidney in a 26-year-old woman with widespread metastasis at initial presentation. The tumor cells showed strong expression for CD99 and FLI-1 monoclonal antibodies and polyclonal antibodies and were negative for WT1 and numerous other markers. The diagnosis was subsequently confirmed by demonstrating t(11;22)(q24;q12) using cytogenetic karyotyping and fluorescence in situ hybridization.

Conclusions

Due to the different prognosis and management between EWS/PNET and other primary renal neoplasms with similar morphology, a histopathologic diagnosis with extreme accuracy should be made. Cytogenetic analysis is an important supportive tool to immunohistochemistry in making the final diagnosis.

Keywords: Ewing sarcoma, Primitive neuroectodermal tumor, Immunohistochemistry, Karyotyping, FISH

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PII: S1092-9134(05)00185-1

doi:10.1016/j.anndiagpath.2005.11.001

Annals of Diagnostic Pathology
Volume 10, Issue 6 , Pages 363-366, December 2006

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