Angiomatoid Fibrous Histiocytoma: An Exceptionally Rare Pulmonary Artery Tumor Presenting as an Asymptomatic Heart Murmur.

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.