Key result
Surgical resection identifies a rare primary cardiac angiomatoid fibrous histiocytoma in a pediatric patient.
Why the study?
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm typically occurring in extremities, with no prior reports of cardiac involvement.
Case Report (n=1)
Answers are grounded in this paper and cite their sources.
This case report identifies a previously unreported cardiac location for angiomatoid fibrous histiocytoma, highlighting the need to consider it in unusual sites.
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 report an unusual case of an 8-year-old boy who presented with progressive exertional dyspnea and fatigue, and was found to have a 4 cm mass in the right atrium. The patient underwent surgical resection. Histologic examination revealed sheets of medium-sized neoplastic cells, with scant cytoplasm, finely stippled chromatin and inconspicuous nucleoli in addition to pseudoangiomatous spaces, and a background of lymphocyte-predominant chronic inflammation. Fluorescence in situ hybridization (FISH) demonstrated EWSR1 gene rearrangement and subsequent Next Generation Sequencing (NGS) identified a EWSR1::ATF1 fusion, confirming the diagnosis of Angiomatoid Fibrous Histiocytoma (AFH). To our knowledge, this represents a previously unreported cardiac location for AFH and highlights the importance of keeping it under diagnostic consideration even in highly unusual sites.
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Jamshed et al. (2026) conducted a case report in Angiomatoid fibrous histiocytoma involving the heart (n=1). Surgical resection was evaluated on Diagnosis and histologic/genetic confirmation. Surgical resection and subsequent genetic testing confirmed a previously unreported cardiac location for angiomatoid fibrous histiocytoma in an 8-year-old boy.
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