Multiple synchronous lung nodules are frequently encountered on computed tomography (CT) scanning of the chest and are most commonly either non-neoplastic or metastases from a known primary malignancy. The finding may initiate a search for primary malignancy elsewhere in the body. An exception to this rule, however, is a class of rare primary lung neoplasms that originate from epithelial (pneumocytes and neuroendocrine), mesenchymal (vascular and meningothelial) and lymphoid tissues of the lung. While these rare neoplasms also present as multiple synchronous unilateral or bilateral lung nodules on chest CT, they are often overlooked in favour of more common causes of multiple lung nodules. The correct diagnosis may be suggested by a multidisciplinary team and established on biopsy, performed either as part of routine diagnostic work-up or staging for malignancy. In this review, we discuss clinical presentations, imaging features, pathology findings and subsequent management of these rare primary neoplasms of the lung.
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