Key Points

• A wide range of rare malignancies can occur as primary pulmonary tumors.
• Most of these lesions have non-specific imaging features that are difficult to distinguish from non–small cell carcinoma.
• Staging is critical.
• When localized, the majority of these lesions should be resected.

Most primary pulmonary malignant neoplasms are bronchogenic carcinomas. The uncommon primary malignant neoplasms that arise in the lung are of disparate histogenesis and include pulmonary blastoma and carcinosarcoma, thymoma,, epithelioid hemangioepitheliomas, melanomas, teratomas, sarcomas, and lymphoreticular disorders. In this chapter, we review the available literature concerning each of these rare malignant neoplasms.