The browser you are using is not supported by this website. All versions of Internet Explorer are no longer supported, either by us or Microsoft (read more here: https://www.microsoft.com/en-us/microsoft-365/windows/end-of-ie-support).

Please use a modern browser to fully experience our website, such as the newest versions of Edge, Chrome, Firefox or Safari etc.

Fusion genes in angiomatoid fibrous histiocytoma

På svenska

Author

Summary, in English

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of low malignant potential and uncertain differentiation. Only three genetically investigated cases of AFH have been reported. Two of them displayed a FUS-ATF1 fusion gene and one showed an EWSR1-ATF1 chimera. Using RT-PCR analysis, we have identified the EWSR1-ATF1 fusion transcript, and delineated the genomic breakpoints, in two new cases of AFH. Previously, the EWSR1-ATF1 fusion protein has been suggested to activate expression of the MITF-M transcript, and therefore the expression pattern of the MITF gene was studied. The MITF-M transcript was not detected in either AFH, in line with the finding that the co-activator SOX10 was not expressed. Thus, of the five AFH that have been molecularly analyzed to date, two have displayed a FUS-ATF1 fusion gene and three have shown an EWSR1-ATF1 chimera. There is no apparent correlation between the type of fusion gene and clinicopathologic features. Nonetheless, RT-PCR for these fusion transcripts remains a valuable diagnostic adjunct in the distinction between AFH and other soft tissue tumors or metastases that may simulate it.

Publishing year

2007-06-18

Language

English

Pages

158-163

Publication/Series

Cancer Letters

Volume

251

Issue

1

Document type

Journal article

Publisher

Elsevier

Topic

  • Cancer and Oncology
  • Medical Genetics

Keywords

  • Activating Transcription Factor 1
  • Base Sequence
  • Bone Neoplasms
  • Calmodulin-Binding Proteins
  • Child
  • Clavicle
  • DNA-Binding Proteins
  • Histiocytoma, Malignant Fibrous
  • Humans
  • Male
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • RNA-Binding Protein FUS
  • RNA-Binding Proteins
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA
  • Spinal Neoplasms
  • Spine
  • Transcription Factors
  • Case Reports
  • Journal Article
  • Research Support, Non-U.S. Gov't

Status

Published

ISBN/ISSN/Other

  • ISSN: 1872-7980