A case of pulmonary artery tumor easily confused with chronic thromboembolic pulmonary hypertension

Abstract Background We present a case of pulmonary artery tumor easily confused with chronic thromboembolic pulmonary hypertension; a treatment previously unreported for such cases. Case presentation A 58-year-old woman was admitted to emergency because of severe heart fatigue and shortness of breath. Echocardiography suggested widening of pulmonary arteries and pulmonary hypertension. She underwent a contrast-enhanced CT scan of the chest, which revealed a filling defect in the left pulmonary artery suspected pulmonary embolism. We performed a mid-thoracic operation under extracorporeal circulation. The left pulmonary artery was cut open, and a tumor-like crystal ball located in the main trunk of the left pulmonary artery, which connected to the wall of the pulmonary artery. The tumor was elliptical, very regular, approximately 2x1.5 cm in size, and had a pedicle connected to the wall of the pulmonary artery. We removed the entire tumor completely, and then we performed a detailed pathological examination, which indicates that the tumor was angiomatous fibrous histocytotoma (AFH). Conclusions AFH is a rare intermediate soft tissue tumor whose diagnosis depends on morphology, immunohistochemistry, and molecular pathology. The treatment is mainly complete surgical resection, and long-term follow-up is required after surgery.