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This case report describes the surgical management of a rare primary angiomatoid fibrous histiocytoma (AFH) in the right pulmonary artery of a 51-year-old female. The tumor was completely resected and the pulmonary artery reconstructed with an artificial vascular graft under cardiopulmonary bypass, including transection of the ascending aorta for optimal exposure. At 5-month follow-up, there was no recurrence or metastasis.
Transection of the ascending aorta during cardiopulmonary bypass can facilitate complete resection and vascular reconstruction in rare pulmonary artery tumors like angiomatoid fibrous histiocytoma.
This report describes an extremely rare case of primary right pulmonary artery angiomatoid fibrous histiocytoma (AFH). A 51-year-old female was admitted with “chest tightness and dizziness for over two months, aggravated by chest pain for three days”. Contrast-enhanced chest CT revealed an irregular filling defect (approximately 2.9 × 1.8 cm) in the right trunk of the pulmonary artery. The tumor in the right pulmonary artery was completely resected, followed by artificial vascular graft replacement under cardiopulmonary bypass with transection of the ascending aorta. Pathological examination confirmed AFH, with fluorescence in situ hybridization showing MDM2 (−, no amplification) and EWSR1 (+, break). No recurrence or metastasis of the tumor was observed during the five-month follow-up. Transection of the ascending aorta under cardiopulmonary bypass provided optimal exposure, enabling complete resection of the tumor while preserving right lung function through artificial vascular graft reconstruction, thereby achieving satisfactory clinical outcomes. This approach offers an effective surgical strategy for the management of pulmonary artery tumors, including AFH.