Comprehensive Review
Primary Pulmonary Lymphoma: Current Status

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Abstract

Primary pulmonary lymphoma (PPL) is a rare disease with a favorable prognosis compared with lung cancer. Although a number of histologic variables of B- and T-cell lymphoma were reported as PPL, marginal zone lymphoma of the mucosa-associated lymphoid tissue type is by far the most frequent diagnosis. This review summarizes the present knowledge of histopathology, molecular biology, diagnosis, prognosis, and treatment of this heterogeneous entity.

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      Primary pulmonary lymphoma (PPL) is defined as an extranodal clonal lymphoid neoplasm involving one or both lungs (parenchyma and/or bronchi) in a patient with no detectable extrapulmonary involvement at diagnosis or during the subsequent 3 months.1 It is a rare clinical entity which accounts for 0.4% of all malignant lymphomas and < 1% of all pulmonary tumors.2, 3 The commonest histologic subtype of PPL (70%-90% of all cases) is the mucosa-associated lymphoid tissue type (MALT) lymphoma (low-grade lymphoma).3, 4

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