Case report: angiosarcoma of the pulmonary artery diagnosed as pulmonary artery emboli

Angiosarcomas are the most common malignant tumors of the heart and great vessels. Late onset and unspecific symptoms are reasons why a diagnosis is made rather late at a time when most tumors have already metastasized. We report a rare case of an angiosarcoma presenting as pulmonary artery emboli.

A 66-year-old patient was initially admitted to the hospital with a STEMI. Days later a successful reanimation due to ventricular fibrillation followed. An emboli of the pulmonary artery was diagnosed as the cause and after a lysis therapy the patient was discharged. A few weeks later the patient suffered an episode of absolute arrhythmia. TTE as well as CT-Scan showed an emboli of the pulmonary artery and a pericardial effusion with compression to the right ventricle. Intraoperative findings, showed an infiltrating tumor of the pulmonary artery, the pulmonary valve, the RVOT, the LA and LV. A resection of the tumor from the pulmonary artery, valve and RVOT was carried out. A new pulmonary valve was implanted with the reconstruction of the RVOT and pulmonary artery. Due to LV infiltration, only a palliative surgical approach was possible. Despite an uncomplicated postoperative course, the patient died at home two months later.

Although a rarity, a tumor of the pulmonary artery should be taken into consideration as a differential diagnosis to pulmonary artery emboli. Development of better diagnostic tools (specific tumor markers) and more effective chemotherapeutic agents is necessary to improve the prognosis of these patients.