Histiocytic proliferations involving the lung span a broad spectrum. Some proliferations are primary; others represent a histiocytic response secondary to conditions in which there may be isolated lung involvement or the lung may be involved as part of a systemic process. Primary histiocytic lung disorders, particularly those of uncertain histogenesis are a heterogeneous and intriguing group of disorders. Although they have been the focus of attention by clinicians and pathologists alike, much is unknown about their etiopathogenesis. Owing to this uncertainty, our understanding of these processes is in a state of flux, and is likely to change as more information is brought to light. This review will focus on pulmonary histiocytic proliferations of uncertain histogenesis. Other histiocytic lesions will be dealt with in brief.
*Department of Pathology, Medical College of Wisconsin, Milwaukee, WI
†Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX
Reprints: R. Nagarjun Rao, MD, FRCPath, Associate Professor, Department of Pathology, Medical College of Wisconsin, Milwaukee, WI 53226 (e-mail: firstname.lastname@example.org).