Phenotypical and molecular distinctness of sinonasal haemangiopericytoma compared to solitary fibrous tumour of the sinonasal tract.

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  • Additional Information
    • Source:
      Publisher: Blackwell Scientific Publications Country of Publication: England NLM ID: 7704136 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2559 (Electronic) Linking ISSN: 03090167 NLM ISO Abbreviation: Histopathology Subsets: MEDLINE
    • Publication Information:
      Original Publication: Oxford, Blackwell Scientific Publications.
    • Subject Terms:
      Biomarkers, Tumor/*metabolism ; Hemangiopericytoma/*pathology ; Nose Neoplasms/*pathology ; Repressor Proteins/*genetics ; STAT6 Transcription Factor/*genetics ; Solitary Fibrous Tumors/*pathology; Adult ; Aged ; Aged, 80 and over ; Female ; Gene Fusion ; Hemangiopericytoma/genetics ; Hemangiopericytoma/metabolism ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Nose Neoplasms/genetics ; Nose Neoplasms/metabolism ; Organ Specificity ; Phenotype ; Repressor Proteins/metabolism ; STAT6 Transcription Factor/metabolism ; Solitary Fibrous Tumors/genetics ; Solitary Fibrous Tumors/metabolism
    • Abstract:
      Aims: Sinonasal haemangiopericytoma (SN-HPC) is a rare sinonasal mesenchymal neoplasm of perivascular myoid cell origin. Solitary fibrous tumour (SFT) occurs only very rarely in the sinonasal tract. SFT and soft tissue HPC have been considered a single entity. Recently, recurrent gene fusions involving NAB2-STAT6 resulting in differential expression of STAT6 were characterized as central molecular events in SFT. However, no data exist for NAB2-STAT6 status or STAT6 expression in SN-HPC.
      Methods and Results: We examined six SN-HPCs and two sinonasal SFTs by immunohistochemistry and RT-PCR for NAB2-STAT6 fusions. SN-HPC affected three females and three males (mean age: 72 years). They expressed smooth muscle actin, lacked strong CD34 reactivity and were negative for nuclear STAT6 expression. RT-PCR analysis confirmed the absence of NAB2-STAT6 fusions in all cases. Conversely, both sinonasal SFTs (in males aged 39 and 52 years) displayed classical features of pleuropulmonary and soft-tissue SFTs (uniformly CD34-positive with strong nuclear expression of STAT6). RT-PCR revealed NAB2-STAT6 fusions in both cases.
      Conclusions: These findings confirm the molecular and phenotypical distinctness of these two entities. While SN-HPC is a site-specific sinonasal neoplasm of as yet unknown molecular pathogenesis, sinonasal SFTs show phenotypical and molecular identity to their pleural/extrapleural counterparts.
      (© 2014 John Wiley & Sons Ltd.)
    • Contributed Indexing:
      Keywords: NAB2; STAT6; gene fusion; head and neck; sinonasal haemangiopericytoma; solitary fibrous tumour
    • Accession Number:
      0 (Biomarkers, Tumor)
      0 (NAB2 protein, human)
      0 (Repressor Proteins)
      0 (STAT6 Transcription Factor)
      0 (STAT6 protein, human)
    • Publication Date:
      Date Created: 20140509 Date Completed: 20170414 Latest Revision: 20170414
    • Publication Date:
      20240829
    • Accession Number:
      10.1111/his.12452
    • Accession Number:
      24807787