Abstract

Marginal zone lymphoma (MZL) is a group of low-grade, indolent, non-Hodgkin lymphomas rarely manifesting in the lungs. A 46-year-old man presenting with shortness of breath and cough was investigated and treated over 10 months for an atypical pattern of lung disease in keeping with cryptogenic organizing pneumonia (COP). Initial lung biopsies were nondiagnostic - repeat sampling eventually showed MZL. Staging whole-body (18F) fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) demonstrated multisystem stage IV disease with intensely avid widespread pulmonary changes resembling COP. The case elegantly illustrates that pulmonary MZL can present insidiously masquerading as COP and shows the value of (18F) FDG PET/CT to stage extranodal MZL.

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