The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts.
Background: Precise diagnosis of small-round-cell tumors is often a challenge to the pathologist and the clinical oncologist. In Ewing's sarcomas and related peripheral primitive neuroectodermal tumors, a t(11;22) translocation or a (21,22) rearrangement is associated with hybrid transcripts of the...
| Publicado en: | New England Journal of Medicine Vol. 331; no. 5; pp. 294 - 300 |
|---|---|
| Autores principales: | , , , , , , , , , , , , , , , , , , , |
| Formato: | research Journal Article |
| Publicado: |
New England Journal of Medicine
8/4/94
|
| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=24919006&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 24919006 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 00284793 NEJ jtl: New England Journal of Medicine issn: 00284793 maglogo: N pubinfo: dt: 8/4/94 vid: 331 iid: 5 pid: 388 pub: New England Journal of Medicine place: Waltham, Massachusetts artinfo: ui: 24919006 24919006 NLM8022439 24919006 10.1056/NEJM199408043310503 NLM8022439 24919006 ppf: 294 ppct: 6 formats: tig: atl: The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. aug: au: Delattre, Olivier Zucman, Jessica Melot, Thomas Garau, Xavier Sastre Zucker, Jean-Michel Lenoir, Gilbert M. Ambros, Peter F. Sheer, Denise Turc-Carel, Claude Triche, Timothy J. Aurias, Alain Thomas, Gilles Delattre, O Zucman, J Melot, T Garau, X S Zucker, J M Lenoir, G M Ambros, P F Sheer, D affil: Laboratoire de Génétique des Tumeurs, INSERM Contrat Jeune Formation, Paris, France sug: subj: Neuroectodermal Tumors, Primitive Diagnosis Bone Neoplasms Diagnosis Osteosarcoma Diagnosis Chromosome Disorders RNA Neuroectodermal Tumors, Primitive, Peripheral Diagnosis Child, Preschool Nucleotides Adolescence Neuroectodermal Tumors, Primitive, Peripheral Human DNA Probes Infant Chromosomes Child Neuroectodermal Tumors, Primitive Polymerase Chain Reaction Adult Bone Neoplasms Osteosarcoma Documentation Validation Studies Comparative Studies Evaluation Research Multicenter Studies Scales Child, Preschool: 2-5 years Adolescent: 13-18 years Infant: 1-23 months Child: 6-12 years Adult: 19-44 years ab: Background: Precise diagnosis of small-round-cell tumors is often a challenge to the pathologist and the clinical oncologist. In Ewing's sarcomas and related peripheral primitive neuroectodermal tumors, a t(11;22) translocation or a (21,22) rearrangement is associated with hybrid transcripts of the EWS gene with the FLI1 or ERG gene. To investigate the diagnostic implication of this observation, we searched for these hybrid transcripts in tumors from patients with clinical and radiologic features of Ewing's sarcoma or peripheral primitive neuroectodermal tumors.Methods: Samples of RNA from 114 tumors were reverse transcribed and subjected to the polymerase chain reaction with primers designed to amplify the relevant chimeric transcripts. All amplified products were sequenced.Results: In-frame hybrid transcripts were observed in 89 cases. A hybrid transcript was found in 83 of 87 cases (95 percent) of Ewing's sarcoma or peripheral primitive neuroectodermal tumors. Samples of RNA from all of 12 tumors that had been proved to be other than Ewing's sarcoma or neuroectodermal tumors had no hybrid transcript. However, 6 of 15 undifferentiated tumors whose type was ambiguous (nonsecreting, poorly differentiated neuroblastoma or undifferentiated sarcoma) contained a hybrid transcript, suggesting that they might have to be reclassified.Conclusions: A subgroup of small-round-cell tumors identified as belonging to the Ewing family of tumors can be defined according to a specific molecular genetic lesion that is detectable by a rapid, reliable, and efficient method. This approach can be applied to small specimens obtained by fine-needle biopsies. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
|---|