Primary Pulmonary Artery Sarcoma: Report of Three Cases and Review of the Literature

Pulmonary artery sarcomas (PASs) are rare tumors of the vasculature of the lung that usually present as a thromboembolism. Failure of anticoagulant therapy to relieve all of a patient's symptoms suggests the diagnosis. Approximately 75% of patients with PAS present with dyspnea, and slightly > 50% also experience chest pain or cough. Imaging studies (chest computed tomography with 3-dimensional reconstruction, magnetic resonance imaging, perfusion lung scan, and pulmonary angiogram) are usually unspecific. A definitive diagnosis requires pathologic examination of tissue obtained by intravascular, percutaneous, or surgical biopsy. Treatment of primary PAS is usually surgical with or without adjuvant chemotherapy or radiation therapy. Because these tumors are rare, data from large randomized trials are not available. Palliative chemotherapy with anthracyclines and ifosfamide is the usual treatment in advanced disease, with response rates of approximately 50%. The mean survival time ranges from 14 months to 18 months. We report 3 cases of PAS treated with surgery and chemotherapy (anthracyclines and ifosfamide) with different outcomes.