MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema

Angiosarcomas (AS) are rare vascular malignancies that arise either de novo as primary tumors or secondary to irradiation or chronic lymphedema. The cytogenetics of angiosarcomas are poorly characterized. We applied array-comparative genomic hybridization as a screening method to identify recurrent...

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Main Authors: Manner, Johanna M. (Author) , Radlwimmer, Bernhard (Author) , Hohenberger, Peter (Author) , Mößinger, Katharina (Author) , Küffer, Stefan (Author) , Sauer, Christian (Author) , Belharazem, Djeda (Author) , Zettl, Andreas (Author) , Coindre, Jean-Michel (Author) , Hallermann, Christian (Author) , Hartmann, Jörg Thomas (Author) , Katenkamp, Detlef (Author) , Katenkamp, Kathrin (Author) , Schöffski, Patrick (Author) , Sciot, Raf (Author) , Wozniak, Agnieszka (Author) , Lichter, Peter (Author) , Marx, Alexander (Author) , Ströbel, Philipp (Author)
Format: Article (Journal)
Language:English
Published: 16 December 2010
In: The American journal of pathology
Year: 2010, Volume: 176, Issue: 1, Pages: 34-39
ISSN:1525-2191
DOI:10.2353/ajpath.2010.090637
Online Access:Resolving-System, lizenzpflichtig, Volltext: https://doi.org/10.2353/ajpath.2010.090637
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S0002944010603227
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Author Notes:Johanna Manner, Bernhard Radlwimmer, Peter Hohenberger, Katharina Mössinger, Stefan Küffer, Christian Sauer, Djeda Belharazem, Andreas Zettl, Jean-Michel Coindre, Christian Hallermann, Jörg Thomas Hartmann, Detlef Katenkamp, Kathrin Katenkamp, Patrick Schöffski, Raf Sciot, Agnieszka Wozniak, Peter Lichter, Alexander Marx, and Philipp Ströbel

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520 |a Angiosarcomas (AS) are rare vascular malignancies that arise either de novo as primary tumors or secondary to irradiation or chronic lymphedema. The cytogenetics of angiosarcomas are poorly characterized. We applied array-comparative genomic hybridization as a screening method to identify recurrent alterations in 22 cases. Recurrent genetic alterations were identified only in secondary but not in primary AS. The most frequent recurrent alterations were high level amplifications on chromosome 8q24.21 (50%), followed by 10p12.33 (33%) and 5q35.3 (11%). Fluorescence in situ hybridization analysis in 28 primary and 33 secondary angiosarcomas (31 tumors secondary to irradiation, 2 tumors secondary to chronic lymphedema) confirmed high level amplification of MYC on chromosome 8q24.21 as a recurrent genetic alteration found exclusively in 55% of AS secondary to irradiation or chronic lymphedema, but not in primary AS. Amplification of MYC did not predispose to high grade morphology or increased cell turnover. In conclusion, despite their identical morphology, secondary AS are genetically different from primary AS and are characterized by a high frequency of high level amplifications of MYC. This finding may have implications both for the diagnosis and treatment of these tumors. 
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