Abstract
Simple SummaryThe group of B-cell lymphomas primarily involving the lung encompasses different histological entities with distinct biological aspects, while sharing some clinical and radiological features related to their common anatomic site of occurrence. Recent molecular advances in the molecular genetics of these lesions have substantially improved of our understanding of the mechanisms of lymphomagenesis, adding novel information to histology in order to better characterize and manage these diseases. This review summarizes the available clinical, radiological, pathological, and molecular data on primary pulmonary B-cell lymphomas, discusses the mechanisms of lymphomagenesis, and highlights the role of a multi-disciplinary management in overcoming the diagnostic and therapeutic challenges in this setting.Primary pulmonary B-cell lymphomas (PP-BCLs) comprise a group of extranodal non-Hodgkin lymphomas of B-cell origin, which primarily affect the lung without evidence of extrapulmonary disease at the time of diagnosis and up to 3 months afterwards. Primary lymphoid proliferations of the lung are most often of B-cell lineage, and include three major entities with different clinical, morphological, and molecular features: primary pulmonary marginal zone lymphoma of mucosa-associated lymphoid tissue (PP-MZL, or MALT lymphoma), primary pulmonary diffuse large B cell lymphoma (PP-DLBCL), and lymphomatoid granulomatosis (LYG). Less common entities include primary effusion B-cell lymphoma (PEL) and intravascular large B cell lymphoma (IVLBCL). A proper workup requires a multidisciplinary approach, including radiologists, pneumologists, thoracic surgeons, pathologists, hemato-oncologists, and radiation oncologists, in order to achieve a correct diagnosis and risk assessment. Aim of this review is to analyze and outline the clinical and pathological features of the most frequent PP-BCLs, and to critically analyze the major issues in their diagnosis and management.
Highlights
Primary pulmonary B-cell lymphoma (PP-BCL) is a rare entity, representing less than 1% of all non-Hodgkin lymphoma (NHL), and 3–4% of all extranodal NHL [1,2,3]
These results suggest a strong association between A. xylosoxidans infection and MALT lymphomagenesis, findings from a Japanese study are contradictory [23], describing a low prevalence of this bacterium in lung biopsies from patients affected by PP-MZL assessed by PCR-based analysis
A number of benign reactive lymphoid processes and B-cell lymphomas enter the differential diagnosis of a PP-MZL, including diffuse lymphoid hyperplasia (DLH)/lymphocytic interstitial pneumonia (LIP), nodular lymphoid hyperplasia (NLH), follicular bronchiolitis, chronic non-specific inflammatory reaction, and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), mantle cell lymphoma (MCL), low-grade follicular lymphoma (FL), lymphoplasmacytic lymphoma (LPL), respectively [52,68]
Summary
Primary pulmonary B-cell lymphoma (PP-BCL) is a rare entity, representing less than 1% of all non-Hodgkin lymphoma (NHL), and 3–4% of all extranodal NHL [1,2,3]. The most frequent entity of PP-BCL is extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (PP-MZL, or MALT lymphoma), with other entities, including other subtypes of low-grade lymphoma, lymphomatoid granulomatosis (LYG) and primary pulmonary diffuse large B cell lymphoma (PP-DLBCL), being far less common [8,9]. PP-BCL involving the pleural cavity, such as primary effusion lymphoma (PEL) is rare, as well as intravascular large B cell lymphoma (IVLBCL) [11] (Table 1). We will discuss the pathogenesis of the most typical B-cell lymphoproliferative disorders primarily involving the lung, namely, PP-MZL/MALT lymphoma, PP-DLBCL, PEL, IVLBCL, and LYG; we will review their epidemiological features, clinical presentation, pathological and molecular aspects, along with prognostic factors and therapeutic options; we will critically analyze the major issues in their diagnosis and management
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