Angiomatoid fibrous histiocytoma: Pleomorphic variant associated with multiplication of EWSR1-CREB1 fusion gene

Tamás Tornóczky, Barna Bogner, Thomas Krausz, Gábor Ottóffy, Károly Szuhai

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor which exceptionally occurs in visceral organs or bones. Histologically this is a bland, monomorphic tumor and only occasionally shows pleomorphism. Vast majority of the soft tissue cases share the same translocation and the resulting EWSR1-CREB1 gene fusion as background pathogenetic alteration. Here we report a 10-year-old boy with subcutaneous tumor of the right shoulder. Histological, immunohistochemical and FISH analyses of the case revealed pleomorphic phenotype, characteristic immunophenotype and multiplication of the EWSR1-CREB1 fusion gene in the nuclei of the tumor cells. The possible explanation of the fusion gene multiplication, its relation to the morphology and the clinical outcome are discussed in the context of the published literature.

Original languageEnglish
Pages (from-to)545-548
Number of pages4
JournalPathology and Oncology Research
Volume18
Issue number2
DOIs
Publication statusPublished - Apr 1 2012

Keywords

  • AFH
  • Angiomatoid fibrous histiocytoma
  • EWSR1-CREB1 fusion gene
  • Multiplication
  • Pleomorphic

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Oncology
  • Cancer Research

Fingerprint Dive into the research topics of 'Angiomatoid fibrous histiocytoma: Pleomorphic variant associated with multiplication of EWSR1-CREB1 fusion gene'. Together they form a unique fingerprint.

  • Cite this