gms | German Medical Science

Objective: Pulmonary artery sarcoma is a rare malignancy which is often mistaken for pulmonary thromboembolism. The presentation is one of central pulmonary artery obstruction and progressive right-heart failure.

Methods: A 43-year-old woman presented to her general practitioner with chronic cough and moderate dyspnea on exertion. She received a symptomatic treatment. Two weeks later she was admitted to a hospital as an emergency with massive dyspnea. A central bilateral pulmonary artery embolism was diagnosed by computed tomography (CT) and was immediately treated with intravenous heparin followed by oral anticoagulation. In the clinical follow-up the CT showed an enlargment of the “thromboembolic mass”. A thrombolysis with rt-PA was unsuccessful. The pulmonary artery mass remained extending from the right ventricular outflow tract to both pulmonary artery branches (Figure 1 [Fig. 1]). The echocardiography showed an increased right ventricular afterload with a systolic pulmonary artery pressure of > 90 mmHg. The patient was referred to our hospital with the presumptive diagnosis of CTEPH for further diagnostic and surgery.

After discussion in our interdisciplinary CTEPH-board, CTEPH was withdrawn and surgery recommended for the assumed diagnosis of pulmonary artery sarcoma. Surgery was performed in September 2012. Via median sternotomy cardio-pulmonary bypass with deep hypothermia was initiated. The left main pulmonary artery was incised and the tumor resected by endarterectomy in an interrupted circulatory arrest time of 23 minutes. A right pneumonectomy was mandatory due to tumor invasion of the media layer of the pulmonary artery. The histopathological examination confirmed the diagnosis of a pulmonary artery sarcoma.

Results: The postoperative course was uneventful. The patient was discharged on post-operative day 12. Nine months postoperatively the patient is alive in reduced clinical condition (ECOG 2), and in complete remission. Adjuvant chemotherapy was recommended but was not performed due to the performance status.

Conclusion: In patients with suspected chronic thromboembolic pulmonary hypertension, these key-points guide to the diagnosis pulmonary artery sarcoma: central pulmonary artery mass, increasing volume despite sufficient anticoagulation and contrast medium uptake. Surgical resection with or without adjuvant chemotherapy currently offers the only chance for long-term survival.