A rare case of angiomatoid fibrous histiocytoma involving the heart: Case report and literature review

Authors:
  • AJAhmed Jamshed
    Ahmed Jamshed
  • IEIsmail Elbaz
    Ismail Elbaz
  • AWAnnikka Weissferdt
    Annikka Weissferdt
  • PMPaari Murugan
    Paari Murugan
  • MGManel Fawzy Gadalla
    Manel Fawzy Gadalla
  • AAAmal A. Alodaini
    Amal A. Alodaini
  • MHMohammad A. Al Hamad
    Mohammad A. Al Hamad
  • TETarek M. ElSharkawy
    Tarek M. ElSharkawy
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Abstract
Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade soft tissue neoplasm which typically occurs in the deep dermis or subcutis of extremities in children and young adults. We present a case of an 8-year-old boy who presented with progressive exertional dyspnea and fatigue, and was found to have a 4 cm right atrial mass. Surgical resection was performed and histology showed neoplastic cells with pseudoangiomatous spaces and mixed lymphocyte predominant chronic inflammation. Fluorescence in situ hybridization (FISH) demonstrated EWSR1 gene rearrangement and subsequent Next Generation Sequencing (NGS) identified a EWSR1::ATF1 fusion consistent with AFH. To our knowledge, this is a previously undescribed location for AFH and highlights the importance of keeping it under diagnostic consideration even in highly unusual sites.