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Peer-reviewedCC BY-NC-ND 4.0JACC Case Reports

Angiomatoid Fibrous Histiocytoma

  • Zulkifl Jafary1
  • Saim Rana1
  • Megan Vaughan1
  • Iraj Hasan1
  • Thomas Ogershok1
  • Erick Trent1
  • Hangyu Watson1
  • Prashanti Atluri2
  • Harshith Thyagaturu3
  1. 1Department of Internal Medicine, West Virginia University, Morgantown, West Virginia, USA
  2. 2Department of Hematology and Oncology, West Virginia University, Morgantown, West Virginia, USA
  3. 3Department of Cardiovascular Disease, West Virginia University, Heart and Vascular Institute, Morgantown, West Virginia, USA
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Abstract

Background: Angiomatoid fibrous histiocytoma (AFH), a type of primary pulmonary artery tumor, is a rare pathology without well-established risk factors, making it a challenging diagnosis.

Case Summary: A 28-year-old man with a history of stage 3 Burkitt lymphoma in remission presented with a new systolic heart murmur. Transthoracic echocardiography noted an indeterminate structure in the main pulmonary artery. Cardiac magnetic resonance imaging and positron emission tomography-computed tomography revealed a 1.9 cm hyperintense mass attached to the pulmonic valve and main pulmonary artery wall. The patient underwent resection of the main pulmonary artery and pulmonic valve with pulmonary artery homograft implantation. Surgical pathology revealed angiomatoid fibrous histiocytoma.

Discussion: AFH is a rare condition with poor outcomes that is often mistaken for pulmonary embolism. Prompt diagnosis and intervention are crucial to improve prognosis.

Take-Home Message: AFH is a rare pathology with poor prognosis that should be considered in all patients with prior oncologic history presenting with a cardiac murmur.

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Cite

BibTeXRIS
BibTeX
@article{jafary2026angiomatoid,
  title = {{Angiomatoid Fibrous Histiocytoma}},
  author = {Zulkifl Jafary and Saim Rana and Megan Vaughan and Iraj Hasan and Thomas Ogershok and Erick Trent and Hangyu Watson and Prashanti Atluri and Harshith Thyagaturu},
  journal = {JACC Case Reports},
  year = {2026},
  month = sep,
  doi = {10.1016/j.jaccas.2026.110028},
  url = {https://doi.org/10.1016/j.jaccas.2026.110028},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Angiomatoid Fibrous Histiocytoma
AU  - Zulkifl Jafary
AU  - Saim Rana
AU  - Megan Vaughan
AU  - Iraj Hasan
AU  - Thomas Ogershok
AU  - Erick Trent
AU  - Hangyu Watson
AU  - Prashanti Atluri
AU  - Harshith Thyagaturu
PY  - 2026
DA  - 2026/09/03
JO  - JACC Case Reports
DO  - 10.1016/j.jaccas.2026.110028
UR  - https://doi.org/10.1016/j.jaccas.2026.110028
AB  - Background: Angiomatoid fibrous histiocytoma (AFH), a type of primary pulmonary artery tumor, is a rare pathology without well-established risk factors, making it a challenging diagnosis.  Case Summary: A 28-year-old man with a history of stage 3 Burkitt lymphoma in remission presented with a new systolic heart murmur. Transthoracic echocardiography noted an indeterminate structure in the main pulmonary artery. Cardiac magnetic resonance imaging and positron emission tomography-computed tomography revealed a 1.9 cm hyperintense mass attached to the pulmonic valve and main pulmonary artery wall. The patient underwent resection of the main pulmonary artery and pulmonic valve with pulmonary artery homograft implantation. Surgical pathology revealed angiomatoid fibrous histiocytoma.  Discussion: AFH is a rare condition with poor outcomes that is often mistaken for pulmonary embolism. Prompt diagnosis and intervention are crucial to improve prognosis.  Take-Home Message: AFH is a rare pathology with poor prognosis that should be considered in all patients with prior oncologic history presenting with a cardiac murmur.
SN  - 2666-0849
ER  -