title: Chimeric EWS-FLI1 transcript in a Ewing cell line with a complex t(11;22;14) translocation.
abstract: Peripheral neuroectodermal tumors include the differentiated neuroepithelioma and the undifferentiated Ewing's tumor. Despite clinical and pathological differences, both malignancies are characterized by a t(11;22)(q24;q12) translocation which is observed in > 90% of the cases. Molecularly, the translocation is underlaid by a rearrangement between the EWS and Fli-1 genes on chromosomes 22 and 11, respectively. Because of the difficulties in the differential diagnosis between various small round cell tumors of childhood, including Ewing's tumor, a molecular diagnostic assay would be desirable. A prerequisite for predicting the reliability of such a test resides in the molecular elucidation of the peripheral neuroectodermal tumor cases which do not exhibit the prototypical translocation. We have analyzed one such case of Ewing's tumor-derived cell line with a t(11;22;14)(q24;q12;q11) translocation. An EWS-Fli-1 fusion transcript was evidenced by polymerase chain reaction amplification of a reverse transcription product obtained from total RNA. Direct sequencing was performed to demonstrate that the molecular rearrangement in this particular Ewing sample resulted in a fusion transcript similar to those observed in tumors with the prototypical translocation.
authors.affiliation: Laboratoire de Biologie des Tumeurs Humaines, CNRS URA 1156, Institut Gustave Roussy, Villejuif, France.
authors.forename: G
authors.initials: G
authors.lastname: Bonin
authors.forename: C
authors.initials: C
authors.lastname: Scamps
authors.forename: C
authors.initials: C
authors.lastname: Turc-Carel
authors.forename: M
authors.initials: M
authors.lastname: Lipinski
mesh_terms: D001483:Base Sequence; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D002892:Chromosomes, Human, Pair 22; D004273:DNA, Neoplasm / Q000737:chemistry*; D004268:DNA-Binding Proteins / Q000737:chemistry* / Q000235:genetics; D006801:Humans; D007621:Karyotyping; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D012333:RNA, Messenger / Q000737:chemistry; D012334:RNA, Neoplasm / Q000737:chemistry; D011993:Recombinant Fusion Proteins / Q000032:analysis; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000235:genetics*; D015534:Trans-Activators / Q000737:chemistry* / Q000235:genetics; D014158:Transcription, Genetic; D014178:Translocation, Genetic / Q000235:genetics*; D014407:Tumor Cells, Cultured
chemical_list: D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D012333:RNA, Messenger; D012334:RNA, Neoplasm; D011993:Recombinant Fusion Proteins; D015534:Trans-Activators