title: EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney.
abstract: Although primitive neuroectodermal tumor (PNET) is a well-recognized entity, its renal localization as a primary site has not been appreciated. Only nine cases of renal PNET exist in the literature. The paucity of renal PNET could be explained by the lack of objective diagnostic techniques that would facilitate its distinction from other primitive round cell tumors of the kidney, such as the more widely recognized monophasic Wilms' tumor and clear-cell sarcoma of the kidney (CCSK), as well as renal carcinoid, or neuroblastoma invading the kidney from the adjacent adrenal gland. The recently identified specific fusion transcripts detectable by reverse transcription polymerase chain reaction (RT-PCR) have provided us with a valuable tool for the detection of renal PNET. This article reports three renal PNET that expressed EWS/FLI-1 fusion transcripts by RT-PCR, in addition to positive staining for MIC2 protein and neuron-specific enolase (NSE). One also exhibited dense core granules in cell processes by electron microscopy. Employment of such methodology will lead to a more accurate classification of renal tumors.
authors.affiliation: Laboratory of Pathology, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: M
authors.initials: M
authors.lastname: Quezado
authors.forename: D R
authors.initials: DR
authors.lastname: Benjamin
authors.forename: M
authors.initials: M
authors.lastname: Tsokos
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D006801:Humans; D007124:Immunoenzyme Techniques; D007680:Kidney Neoplasms / Q000737:chemistry* / Q000235:genetics / Q000473:pathology; D008297:Male; D017599:Neuroectodermal Tumors / Q000737:chemistry* / Q000235:genetics / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000032:analysis* / Q000235:genetics; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D016133:Polymerase Chain Reaction; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D012194:RNA-Directed DNA Polymerase; D014157:Transcription Factors / Q000032:analysis* / Q000235:genetics
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors; D012194:RNA-Directed DNA Polymerase; D010751:Phosphopyruvate Hydratase