Pathology & Oncology Research

, Volume 18, Issue 2, pp 545–548

Angiomatoid Fibrous Histiocytoma: Pleomorphic Variant Associated with Multiplication of EWSR1-CREB1 Fusion Gene

  • Tamás Tornóczky
  • Barna Bogner
  • Thomas Krausz
  • Gábor Ottóffy
  • Károly Szuhai
Communication

DOI: 10.1007/s12253-011-9468-6

Cite this article as:
Tornóczky, T., Bogner, B., Krausz, T. et al. Pathol. Oncol. Res. (2012) 18: 545. doi:10.1007/s12253-011-9468-6

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.

Keywords

AFH Angiomatoid fibrous histiocytoma Pleomorphic Multiplication EWSR1-CREB1 fusion gene 

Copyright information

© Arányi Lajos Foundation 2011

Authors and Affiliations

  • Tamás Tornóczky
    • 1
  • Barna Bogner
    • 2
  • Thomas Krausz
    • 3
  • Gábor Ottóffy
    • 4
  • Károly Szuhai
    • 5
  1. 1.Department of Pathology, II. Diagnostic Laboratory, Faculty of Medicine, Clinical CenterPécs UniversityPécsHungary
  2. 2.Department of PathologyBalassa János County HospitalSzekszárdHungary
  3. 3.Department of Anatomic and Surgical PathologyUniversity of ChicagoChicagoUSA
  4. 4.Department of Pediatrics, Oncohaematology Division, Faculty of Medicine, Clinical CenterPécs UniversityPécsHungary
  5. 5.Department of Molecular Cell BiologyLeiden University Medical CenterLeidenThe Netherlands

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