Primary pulmonary valve sarcoma involving pulmonary artery and right ventricular outflow tract

The group of pulmonary valve and pulmonary artery primary tumors is the most frequently represented by schwannomas, myxomas, papillary fibroelastomas, primary choriocarcinomas and sarcomas. These tumors are very rare. The most common clinical manifestation of pulmonary artery tumor is dyspnea, followed by chest pain, cough, and haemoptysis.A case of 44-year-old male with history of progressive dyspnea, fever, cough, and weight loss is presented. Imaging methods showed large saddle embolus in the right ventricle outflow tract, pulmonary valve and pulmonary artery trunk. He was admitted to a hospital for anticoagulation therapy. Since there was no clinical improvement, the patient had to undergo surgery. Nearly full artery caliber filling tumor in pulmonary artery was found with its origin in pulmonary valve. Next exploration showed several little tumors in right ventricle outflow tract and also in pulmonary artery. The final outcome of histological examination showed the presence of leiomyosarcoma high grade 3.Presented case highlights that pulmonary artery tumor should always be included in the differential diagnosis of pulmonary embolism, especially if the symptoms progress while on adequate anticoagulation, and if any risk factor for deep vein thrombosis is not present. Unfortunately, prognosis of pulmonary artery sarcomas is usually dismal, secondary to late diagnosis.