Pulmonary artery sarcomas. A review and report of a case.

We examined a case of primary pulmonary artery sarcoma and reviewed 45 cases that we found previously reported in the English literature, with particular attention given to the pathologic features. These sarcomas involve the pulmonary arterial trunk, left and right main pulmonary arteries, pulmonary valve, and right ventricular outflow tract. They have prominent intravascular growth along the arterial intima. Lung involvement commonly occurs by direct extension through the pulmonary vasculature or by distant metastases. These sarcomas contain a variety of heterologous components and areas of bone, cartilage, and fibrous tissue may be remarkably well-differentiated. Management of these sarcomas will be aided by an understanding of their biologic behavior and pathologic features.