title: Extranodal Marginal Zone Lymphoma of the Lung: Evolution from an Underlying Reactive Lymphoproliferative Disorder.
abstract: Extranodal Marginal Zone Lymphoma (ENMZL) of Mucosa-Associated Lymphoid Tissue (MALT) is a problematic and sometimes controversial diagnosis. While commonly seen in the stomach in the setting of chronicHelicobacter pyloriinfection, other extranodal sites, such as the lung, may also present with disease. ENMZL is clinically and morphologically heterogeneous; however, regardless of presentation, the etiology lies in the accumulation of lymphoid tissue in non-traditional sites. This phenomenon is typically secondary to an underlying inflammatory stimulus such as chronic infection or autoimmune states. The current case report details the clinical history of a patient with Sjögren syndrome over a four year period who eventually developed ENMZL. The patient initially presented with an atypical, but polyclonal, lymphoproliferative process diagnosed as lymphocytic interstitial pneumonia. Over time, the patient showed evolution to a monoclonal process with associated radiologic progression of disease. This evolution manifested as a dense lymphoid infiltrate with prominent plasmacytic differentiation and the development of a lung mass radiologically. This case contributes to the growing body of knowledge that suggests ENMZL lies along a biological spectrum of lymphoproliferative disorders whereby a benign, reactive process may eventually undergo malignant transformation. This evolution likely represents the acquisition of genetic abnormalities that allow autonomous proliferation in the absence of the initial immune stimulus. In practice, determining when this event occurs and, thus, distinguishing between reactive and neoplastic disorders within this spectrum may be difficult as no single clinicopathologic feature may be present to establish the diagnosis. This case further illustrates the importance of correlating the clinical, radiologic and pathologic data to evaluate patients with atypical pulmonary lymphoproliferative disorders and to allow the optimal management of their disease.
authors.affiliation: Department of Pathology, West Virginia University School of Medicine, USA.
authors.forename: Jon Nicholas
authors.initials: JN
authors.lastname: Rubenstein
authors.affiliation: Department of Pathology, West Virginia University School of Medicine, USA.
authors.forename: Colleen
authors.initials: C
authors.lastname: Beatty
authors.affiliation: Department of Pathology, West Virginia University School of Medicine, USA.
authors.forename: Zoe
authors.initials: Z
authors.lastname: Kinkade
authors.affiliation: Department of Radiology, West Virginia University School of Medicine, USA.
authors.forename: Cara
authors.initials: C
authors.lastname: Bryan
authors.affiliation: Department of Radiology, West Virginia University School of Medicine, USA.
authors.forename: Jeffery Paul
authors.initials: JP
authors.lastname: Hogg
authors.affiliation: Professor of Microbiology, Immunology and Cell Biology, Mary Babb Randolph Cancer Center at West Virginia University School of Medicine.
authors.forename: Laura F
authors.initials: LF
authors.lastname: Gibson
authors.affiliation: Department of Pathology, West Virginia University School of Medicine, USA.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Vos
keywords: Atypical lymphoplasmacytic infiltrate; Extranodal marginal zone lymphoma; Lymphocytic interstitial pneumonia; MALT lymphoma; Sjögren syndrome