title: Expression of WT1 in pediatric small cell tumors: report of two cases with a possible mesothelial origin.
abstract: The WT1 gene is normally expressed in fetal kidney and mesothelium, and its expression has been suggested as a marker for Wilms tumor and mesothelioma. We examined WT1 expression levels by reverse-transcriptase polymerase chain reaction (RT-PCR) in 38 childhood small-cell tumors including Wilms tumor, embryonal and alveolar rhabdomyosarcoma, Ewing sarcoma, lymphoma, desmoplastic small round-cell tumor (DSRCT), synovial sarcoma, extrarenal rhabdoid tumor, and two tumors that were atypical for this group of tumors. WT1 expression was only detected in Wilms tumor, rhabdoid tumor, and in these two cases of uncertain histogenesis. Both arose in the peritoneal cavity and by immunohistochemistry were diffusely positive for vimentin, keratin, and desmin. Tonofilaments were identified by electron microscopy in one of the cases. RT-PCR failed to detect the t(11;22) translocation associated with DSRCT in either case. Our results suggest that WT1 expression is an unusual feature of childhood non-Wilms tumors and, in the right setting, it may indicate a mesothelial origin. The expression of WT1 may play a role in mesodermal cells acquiring epithelial characteristics, a concept supported by the mixed epithelial and mesenchymal phenotype of these two cases.
authors.affiliation: Department of Pediatric Laboratory Medicine, Hospital for Sick Children Toronto, Ontario, Canada.
authors.forename: P
authors.initials: P
authors.lastname: Thorner
authors.forename: J
authors.initials: J
authors.lastname: Squire
authors.forename: N
authors.initials: N
authors.lastname: Plavsic
authors.forename: R
authors.initials: R
authors.lastname: Jong
authors.forename: M
authors.initials: M
authors.lastname: Greenberg
authors.forename: M
authors.initials: M
authors.lastname: Zielenska
mesh_terms: D014408:Biomarkers, Tumor; D018288:Carcinoma, Small Cell / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D002675:Child, Preschool; D002880:Chromosomes, Human, Pair 11; D004268:DNA-Binding Proteins / Q000096:biosynthesis* / Q000235:genetics; D004848:Epithelium / Q000473:pathology; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008223:Lymphoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008297:Male; D008654:Mesothelioma / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D016133:Polymerase Chain Reaction; D012208:Rhabdomyosarcoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D012512:Sarcoma, Ewing / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D013736:Testicular Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D014157:Transcription Factors / Q000096:biosynthesis* / Q000235:genetics; D025721:WT1 Proteins; D009396:Wilms Tumor / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D016335:Zinc Fingers
chemical_list: D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; D014157:Transcription Factors; D025721:WT1 Proteins