ABSTRACT
Primary tumors of the pulmonary vasculature are extremely rare (1) and may affect the pulmonary arteries or the pulmonary veins. Although there are some benign neoplasms (i.e., myxomas, leiomyomas, fibroelastomas), the majority of these tumors are malignant, sarcomatous neoplasms (2). Primary sarcomas of the lung represent only 1 in every 500 primary lung cancers, but are a little more frequent when looking at nonepithelial primary lung cancers (1). Primary soft tissue sarcomas of the lung are divided into 3 categories based on site of origin: parenchymal sarcomas, small-vessel sarcomas, and large-vessel sarcomas (3). This chapter focuses on largevessel sarcomas.
