Key result
Surgical resection of a pulmonary artery mass reveals angiomatoid fibrous histiocytoma in a 28-year-old man.
Why the study?
Angiomatoid fibrous histiocytoma is a rare primary pulmonary artery tumor without well-established risk factors, making it a challenging diagnosis.
Case Report (n=1)
Angiomatoid fibrous histiocytoma is a rare primary pulmonary artery tumor that should be considered in patients with prior oncologic history presenting with a cardiac murmur.
Rare pulmonary artery masses merit consideration in young adults with prior malignancy and murmurs; leaves open questions on tumor associations and surveillance.
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.
No takes yet. Share an insight, caveat, or question.
Jafary et al. (2026) conducted a case report in Angiomatoid fibrous histiocytoma (n=1). Surgical resection with pulmonary artery homograft implantation was evaluated. Surgical resection of a 1.9 cm mass in the main pulmonary artery revealed angiomatoid fibrous histiocytoma in a 28-year-old man with a history of Burkitt lymphoma.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: