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Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma
Department of Clinical Genetics, University Hospital, SE-221 85 Lund, Sweden.
Department of Clinical Genetics, University Hospital, SE-221 85 Lund, Sweden.
Department of Clinical Genetics, University Hospital, SE-221 85 Lund, Sweden.
Department of Pathology, Sahlgrenska University Hospital, Gothenburg, Sweden.
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2005 (English)In: Genes, Chromosomes and Cancer, ISSN 1045-2257, Vol. 44, no 1, 97-102 p.Article in journal (Refereed) Published
Abstract [en]

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that usually occurs in children and young adults. Only two cases of AFH with genetic rearrangements have been reported previously, both of these had a FUS-ATF1 fusion gene. We have studied an AFH from a 9-year-old boy whose tumor displayed a t(12,22)(q13,q12) as the sole cytogenetic aberration. FISH, RT-PCR, and sequence analyses revealed an EWSR1-ATF1 fusion gene that has previously been reported in clear cell sarcoma (CCS), a soft tissue sarcoma that is morphologically and clinically distinct from AFH. This study thus has demonstrated that the EWSR1-ATF1 chimera represents a fusion gene that can be associated with different tumor types. Simultaneous expression of the EWSR1-ATF1 and MITF-M transcripts in CCS has led to the proposal that the MITF-M promoter is transactivated by EWSR1-ATF1. The AFH, however, did not express the MITF-M transcript, supporting the theory that MITF-M expression in CCS is a reflection of its cellular origin, rather than a consequence of the presence of an EWSR1-ATF1 fusion protein. Activation of the EWSR1-ATF1 oncogene is probably an early step in the transformation process, but the overall gene expression patterns are likely to vary considerably between AFH and CCS, in keeping with their clinicopathologic differences. © 2005 Wiley-Liss, Inc.

Place, publisher, year, edition, pages
2005. Vol. 44, no 1, 97-102 p.
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Medical and Health Sciences
URN: urn:nbn:se:liu:diva-50429DOI: 10.1002/gcc.20201OAI: diva2:271325
Available from: 2009-10-11 Created: 2009-10-11 Last updated: 2011-01-12

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Kalén, Anders
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Faculty of Health SciencesOrthopaedics and Sports Medicine Department of Orthopaedics Linköping
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