WT1 staining reliably differentiates desmoplastic small round cell tumor from ewing sarcoma/primitive neuroectodermal tumor: An immunohistochemical and molecular diagnostic study

D. A. Hill, J. D. Pfeifer, E. F. Marley, L. P. Dehner, P. A. Humphrey, X. Zhu, P. E. Swanson

Research output: Contribution to journalArticlepeer-review

130 Scopus citations

Abstract

Differentiating desmoplastic small round cell tumor (DSRCT) from another similar small round cell tumor of childhood, the Ewing sarcoma/primitive neuroectodennal tumor (EWS/PNET), can be difficult because morphologic and immunohistochemical features overlap. We studied the predictive value of immunohistochemistry with an antibody to the C-terminal region of the Wilms tumor (WT1) protein for differentiating DSRCT from EWS/PNET in 24 malignant small round cell tumors that had been previously diagnosed as DSRCT or EWS/PNET by standard methods. We performed reverse transcriptase-polymerase chain reaction (RT-PCR) analysis in cases with available tissue as a confirmatory measure: 6 of 13 DSRCTs were informative by RT-PCR, and 6 of 6 showed an EWS-WT1 fusion; all 13 DSRCTs showed strong, definitive nuclear staining with the WT1 antibody. All 11 EWS/PNETs were WT1 antibody negative; 7 of 11 cases classified as EWS/PNET were informative by RT-PCR, and 7 of 7 showed an EWS-FLI-1 fusion. For cases in which the morphologic and immunohistochemical features are consistent with a diagnosis of DSRCT, WT1 antibody staining predicts the EWS-WT1 translocation with high sensitivity and specificity and is, therefore, useful for differentiating DSRCT from EWS/PNET when genetic information is unavailable.

Original languageEnglish
Pages (from-to)345-353
Number of pages9
JournalAmerican journal of clinical pathology
Volume114
Issue number3
DOIs
StatePublished - 2000

Keywords

  • Desmoptastic small round cell tumor
  • Ewing sarcoma
  • Fusion protein
  • Immunohistochemistry
  • Molecular diagnostics
  • Primitive neuroectodermal tumor
  • Wilms tumor gene (WT1)

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