title: Desmoplastic small round cell tumor: RT-PCR analysis and immunohistochemical detection of the Wilm's tumor gene WT1.
abstract: Desmoplastic small round cell tumor is an aggressive neoplasm first described in 1991. Recently, a reciprocal translocation t(11;22)(p13;q12) has been characterized by conventional cytogenetic studies and molecular analysis. This translocation involves the Ewing's sarcoma gene on chromosome 22 and the Wilms' tumor gene WT1 on chromosome 11. The chimeric transcript corresponding to the fusion gene could be detected by the reverse transcriptase-polymerase chain reaction (RT-PCR). Using an anti-WT1 antibody, the WT1 part of the putative chimeric protein could be recognized by immunohistochemistry. We describe two well-characterized cases of intraabdominal desmoplastic small round cell tumor in two male patients aged 14 and 28 with both RT-PCR analysis and immunostaining for WT1. In this report, we insist on the necessity to increase the RT-PCR analysis in DSRCT in order to obtain a precise differential diagnosis. In addition, WT1 immunostaining may serve as a useful diagnostic marker for DSRCT.
authors.affiliation: Département de Pathologie, Centre Hospitalier Universitaire, Grenoble, France.
authors.forename: R
authors.initials: R
authors.lastname: Barnoud
authors.forename: O
authors.initials: O
authors.lastname: Delattre
authors.forename: M
authors.initials: M
authors.lastname: Péoc'h
authors.forename: D
authors.initials: D
authors.lastname: Pasquier
authors.forename: D
authors.initials: D
authors.lastname: Plantaz
authors.forename: D
authors.initials: D
authors.lastname: Leroux
authors.forename: B
authors.initials: B
authors.lastname: Pasquier
mesh_terms: D000008:Abdominal Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D000293:Adolescent; D000328:Adult; D018288:Carcinoma, Small Cell / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D017931:DNA Primers / Q000737:chemistry; D004273:DNA, Neoplasm / Q000032:analysis; D004268:DNA-Binding Proteins / Q000378:metabolism*; D003893:Desmin / Q000378:metabolism*; D017809:Fatal Outcome; D016162:Genes, Wilms Tumor / Q000235:genetics*; D006801:Humans; D007124:Immunoenzyme Techniques; D007621:Karyotyping; D008297:Male; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014157:Transcription Factors / Q000378:metabolism*; D025721:WT1 Proteins
chemical_list: D017931:DNA Primers; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D003893:Desmin; D014157:Transcription Factors; D025721:WT1 Proteins