Extranodal Marginal Zone Lymphoma: No Longer Just a Sidekick

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice.A 51-year-old healthy female with good performance status presented for gynecologic surgery for a benign condition. A preprocedure chest x-ray showed a right lower lobe infiltrate. A subsequent computed tomography (CT) scan of the chest with contrast revealed a large consolidative right lower lobe mass with surrounding inflammation (Fig 1A). Bronchoscopy with biopsy revealed a low-grade lymphoma with the following immunophenotype: CD45(+), CD20(+), BCL2(+), CD10 negative, CD5 negative, cyclin D1 negative, and Ki-67 index of less than 5%. Morphology and immunohistochemistry were most consistent with pulmonary extranodal marginal zone lymphoma (ENMZL; Fig 2). The patient was asymptomatic and denied fevers, sweats, weight loss, shortness of breath or dyspnea on exertion, or cough. Her history was notable for exposure to parrots over several months before presentation. Complete staging with a CT of the chest, abdomen, and pelvis with contrast redemonstrated disease that was localized to the chest with mild compression of the pulmonary vasculature but no other evidence of lymphoma. She was referred to discuss management of stage I-AE pulmonary ENMZL lymphoma.