| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
Molecular Biology and Genetics |
Medical Research Council Cancer Cell Unit, Hutchison/MRC Research Centre, Cambridge, CB2 2XZ [D. C. S., N. C.]; West Midlands Regional Cytogenetics Laboratory, Birmingham Women’s Hospital, Birmingham, B15 2TG [D. J. M.]; Departments of Cytogenetics [P. R.] and Pathology [C. C.], St. James’s University Hospital, Leeds, LS9 7TF; Cytogenetics, Department of Medical Genetics [K. S.] and Department of Paediatric Oncology [J. N.], Addenbrooke’s NHS Trust, Cambridge, CB2 2QQ; University of Cambridge/Cancer Research United Kingdom Department of Oncology, Cambridge, CB2 2XZ [S-F. C.]; The Royal Orthopaedic Hospital NHS Trust, Bristol Road South, Birmingham, B31 2AP [R. M. T.]; and Department of Pathology, Birmingham Children’s Hospital, Birmingham, B4 6NH [P. R.], United Kingdom
Ewing’s tumors are rare pediatric neoplasms that are characterized by specific chromosomal translocations and gene rearrangements. All of the fusion genes reported to date in Ewing’s tumors juxtapose the EWS gene at 22q12 to an ETS-related gene, the most common of which are FLI1 at 11q24 and ERG at 21q22. We present here four cases of Ewing’s tumor, which showed no evidence of a EWS gene rearrangement, but instead contained translocations involving 16p11 and 21q22. A rearrangement involving the same chromosome bands, t(16;21)(p11;q22), is found in rare cases of acute myeloid leukemia and fuses the FUS gene at 16p11 to the ERG gene at 21q22. In two of our Ewing’s tumor cases, we were able to show at the sequence level that the translocation between chromosomes 16 and 21 similarly results in a FUS/ERG fusion. In one case, exons 1–5 and most of exon 6 of FUS were fused in-frame to exon 9 of ERG; in the other case, FUS exons 1–7 were fused in-frame to ERG exons 8–9. The functional fusion transcript is expected to be expressed from the der(21)t(16;21) derivative. In the two other t(16;21)-positive Ewing’s cases, we performed bacterial artificial chromosome fluorescence in situ hybridization analysis on metaphases and interphase nuclei to demonstrate colocalization of bacterial artificial chromosomes containing FUS and ERG genes, also highly suggestive of fusion gene formation. These represent the first four cases where FUS, rather than EWS, is rearranged with an ETS-family transcription factor in Ewing’s tumors. Our data provide additional evidence that the transactivation domains of the TET family of RNA-binding proteins (such as EWS and FUS) are interchangeable, and suggests a novel mechanism of oncogenesis in Ewing’s tumors.
This article has been cited by other articles:
|
|
 |
|
  M V Shingde, M Buckland, K J Busam, S W McCarthy, J Wilmott, J F Thompson, and R A Scolyer Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis J. Clin. Pathol., October 1, 2009; 62(10): 915 - 919. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Szuhai, M. IJszenga, D. de Jong, A. Karseladze, H. J. Tanke, and P. C.W. Hogendoorn The NFATc2 Gene Is Involved in a Novel Cloned Translocation in a Ewing Sarcoma Variant That Couples Its Function in Immunology to Oncology Clin. Cancer Res., April 1, 2009; 15(7): 2259 - 2268. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Rapaport, E. Barillot, and J.-P. Vert Classification of arrayCGH data using fused SVM Bioinformatics, July 1, 2008; 24(13): i375 - i382. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Liu, H. Cheng, W. Kwan, J. M. Lubieniecka, and T. O. Nielsen Histone deacetylase inhibitors induce growth arrest, apoptosis, and differentiation in clear cell sarcoma models Mol. Cancer Ther., June 1, 2008; 7(6): 1751 - 1761. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Rossi, K. Szuhai, M. Ijszenga, H. J. Tanke, L. Zanatta, R. Sciot, C. D.M. Fletcher, A. P. Dei Tos, and P. C.W. Hogendoorn EWSR1-CREB1 and EWSR1-ATF1 Fusion Genes in Angiomatoid Fibrous Histiocytoma Clin. Cancer Res., December 15, 2007; 13(24): 7322 - 7328. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. C. Torchia, K. Boyd, J. E. Rehg, C. Qu, and S. J. Baker EWS/FLI-1 Induces Rapid Onset of Myeloid/Erythroid Leukemia in Mice Mol. Cell. Biol., November 15, 2007; 27(22): 7918 - 7934. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. L. Ng, M. J. O'Sullivan, C. J. Pallen, M. Hayes, P. W. Clarkson, M. Winstanley, P. H.B. Sorensen, T. O. Nielsen, and D. E. Horsman Ewing Sarcoma with Novel Translocation t(2;16) Producing an In-Frame Fusion of FUS and FEV J. Mol. Diagn., September 1, 2007; 9(4): 459 - 463. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. G. Barr and R. B. Womer Molecular Diagnosis of Ewing Family Tumors: Too Many Fusions... ? J. Mol. Diagn., September 1, 2007; 9(4): 437 - 440. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Wang, R. Bhargava, T. Zheng, L. Wexler, M. H. Collins, D. Roulston, and M. Ladanyi Undifferentiated Small Round Cell Sarcomas with Rare EWS Gene Fusions: Identification of a Novel EWS-SP3 Fusion and of Additional Cases with the EWS-ETV1 and EWS-FEV Fusions J. Mol. Diagn., September 1, 2007; 9(4): 498 - 509. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 X. Gu, L. F. Zerbini, H. H. Otu, M. Bhasin, Q. Yang, M. G. Joseph, F. Grall, T. Onatunde, R. G. Correa, and T. A. Libermann Reduced PDEF Expression Increases Invasion and Expression of Mesenchymal Genes in Prostate Cancer Cells Cancer Res., May 1, 2007; 67(9): 4219 - 4226. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. G. Barr and P. J. Zhang The impact of genetics on sarcoma diagnosis: an evolving science. Clin. Cancer Res., September 15, 2006; 12(18): 5256 - 5257. [Full Text] [PDF]
|
|
|
|
|
|
G. Ng, J. Huang, I. Roberts, and N. Coleman Defining Ploidy-Specific Thresholds in Array Comparative Genomic Hybridization to Improve the Sensitivity of Detection of Single Copy Alterations in Cell Lines J. Mol. Diagn., September 1, 2006; 8(4): 449 - 458. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Kawamura-Saito, Y. Yamazaki, K. Kaneko, N. Kawaguchi, H. Kanda, H. Mukai, T. Gotoh, T. Motoi, M. Fukayama, H. Aburatani, et al. Fusion between CIC and DUX4 up-regulates PEA3 family genes in Ewing-like sarcomas with t(4;19)(q35;q13) translocation Hum. Mol. Genet., July 1, 2006; 15(13): 2125 - 2137. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Bernstein, H. Kovar, M. Paulussen, R. L. Randall, A. Schuck, L. A. Teot, and H. Juergensg Ewing's Sarcoma Family of Tumors: Current Management. Oncologist, May 1, 2006; 11(5): 503 - 519. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. J. Law, K. L. Cann, and G. G. Hicks TLS, EWS and TAF15: a model for transcriptional integration of gene expression Brief Funct Genomic Proteomic, March 1, 2006; 5(1): 8 - 14. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Zou, H. Ichikawa, M. L. Blackburn, H.-M. Hu, A. Zielinska-Kwiatkowska, Q. Mei, G. J. Roth, H. A. Chansky, and L. Yang The Oncogenic TLS-ERG Fusion Protein Exerts Different Effects in Hematopoietic Cells and Fibroblasts Mol. Cell. Biol., July 15, 2005; 25(14): 6235 - 6246. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H.-Y. Huang, P. B. Illei, Z. Zhao, M. Mazumdar, A. G. Huvos, J. H. Healey, L. H. Wexler, R. Gorlick, P. Meyers, and M. Ladanyi Ewing Sarcomas With p53 Mutation or p16/p14ARF Homozygous Deletion: A Highly Lethal Subset Associated With Poor Chemoresponse J. Clin. Oncol., January 20, 2005; 23(3): 548 - 558. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Cancer Research | Clinical Cancer Research |
| Cancer Epidemiology Biomarkers & Prevention | Molecular Cancer Therapeutics |
| Molecular Cancer Research | Cancer Prevention Research |
| Cancer Prevention Journals Portal | Cancer Reviews Online |
| Annual Meeting Education Book | Meeting Abstracts Online |
