[Leiomyosarcoma of pulmonary artery origin diagnosed preoperatively].

A 59-year-old woman was pointed out isotope uptake at the right pulmonary hilum by positron emission tomography/computed tomography (PET/CT) performed for postoperative follow up of thyroid cancer. Chest enhanced CT showed a mass in the right pulmonary artery, which was diagnosed as sarcoma of pulmonary artery origin by a transcatheter biopsy. Chemotherapy by doxorubicin with anticoagulation therapy was started 1st due to the secondary pulmonary hypertension. Pulmonary hypertension was improved, but the size of the tumor enlarged. She underwent right pneumonectomy with mediastinal dissection. The tumor was completely resected, but brain metastases developed. Postoperative pathological diagnosis was leiomyosarcoma. A rare case of preoperatively diagnosed pulmonary arterial sarcoma was reported.