Primary Pulmonary Lymphomas

Primary lung lymphoma (PLL) is a rare disease that comprises <0.5% of all primary lung tumors. It is defined as lymphoma confined to the lung with or without hilar lymph node involvement at the time of diagnosis or up to 3 months thereafter. Patients with PLL may be asymptomatic or manifest nonspecific clinical symptoms, for example, cough, chest pain, and dyspnea. Some individuals may be immunosupressed or have an autoimmune disorder. Radiologically, PLL can mimic pneumonia, lung carcinoma, or metastasis, and therefore, histologic confirmation is mandatory for definitive diagnosis. Primary lung marginal zone lymphoma of mucosa-associated lymphoid tissue type comprises 70% to 80% of cases. Less common B-cell lymphomas include diffuse large B-cell lymphoma, lymphomatoid granulomatosis (LyG), plasmacytoma, and other small lymphocytic lymphomas. PLLs of T-cell origin, largely represented by anaplastic large cell lymphoma, are extremely rare. LyG is an Epstein-Barr virus (EBV)-driven B-cell lymphoid neoplastic proliferation rich in T cells that produces vasculitis. The disease may present at different stages of progression. Differential diagnosis of PLL varies according to the lymphoma subtype: pulmonary mucosa-associated lymphoid tissue lymphoma should be distinguished from reactive inflammatory conditions, whereas high-grade lymphomas may resemble poorly differentiated lung carcinoma, metastatic disease, and other lymphomas. LyG can resemble inflammatory, infectious, and other lymphoid neoplastic processes. A panel of immunohistochemical markers, flow cytometry, and molecular methods are necessary to confirm the diagnosis in the majority of cases. In this article we review the clinical, radiologic, pathologic, and molecular characteristics of several B-cell and T-cell PLLs with exception of Hodgkin lymphoma and posttransplant lymphoproliferative disorder.