# Case Report: Right pulmonary artery resection with artificial vascular graft replacement under cardiopulmonary bypass for pulmonary artery angiomatoid fibrous histiocytoma

## TL;DR

A rare case of a tumor in the right pulmonary artery was successfully treated with surgery and graft replacement, preserving lung function.

## Contribution

This case report presents a novel surgical approach for treating pulmonary artery angiomatoid fibrous histiocytoma.

## Key findings

- Complete tumor resection and vascular graft replacement under cardiopulmonary bypass achieved no recurrence in five months.
- Transection of the ascending aorta provided optimal exposure for successful tumor removal.
- Pathological and genetic analysis confirmed the diagnosis of AFH with no MDM2 amplification.

## Abstract

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.