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Pulmonary Lymphoma
Sonia L. Betancourt-Cuellar, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Primary pulmonary lymphoma (PPL)
                • Non-Hodgkin lymphoma (NHL)
                  • Mucosa-associated lymphoid tissue (MALT) lymphoma
                  • Diffuse large B-cell lymphoma (DLBCL)
                  • Lymphomatoid granulomatosis (LG)
                • Hodgkin lymphoma (HL)
              • Secondary pulmonary lymphoma (SPL)
            • Synonyms

              • Low-grade primary pulmonary B-cell lymphoma
              • MALT lymphoma
            • Definitions

              • PPL
                • Pulmonary clonal lymphoid proliferation in patient with no detectable extrapulmonary lymphoma for at least 3 months after initial diagnosis
                • Represents 0.5% of primary lung malignancies
                • Almost all cases of PPL are NHL
                  • ~ 70 cases of primary pulmonary HL reported
                • Represents only 3-4% of cases of primary extranodal NHL
                • Most common subtypes: MALT lymphoma, high-grade DLBCL, LG
                  • MALT lymphoma: Low-grade extranodal B-cell lymphoma, represents majority of cases of PPL
                • Uncommon subtypes: Pulmonary intravascular lymphoma, primary pulmonary plasmacytoma
              • SPL
                • Common as manifestation of systemic involvement by HL and NHL
                • Lung involvement rate higher in HL (85%) than in NHL (24%), but NHL is far more common and represents up to 90% of all SPL
                • Mechanisms of lung involvement
                  • Invasion from hilar or mediastinal lymph nodes
                  • Hematogeneous or lymphatic dissemination

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Demographics

                                • Mucosa-Associated Lymphoid Tissue Lymphoma

                                  • High-Grade B-Cell Non-Hodgkin Lymphoma

                                    • Lymphomatoid Granulomatosis

                                      • Primary Pulmonary Plasmacytoma

                                        • Pulmonary Intravascular Lymphoma

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            Selected References

                                            1. Angirish B et al: Pulmonary manifestations of lymphoma: A pictorial essay. Lung India. 37(3):263-7, 2020
                                            2. Cozzi D et al: Primary pulmonary lymphoma: imaging findings in 30 cases. Radiol Med. 124(12):1262-9, 2019
                                            3. Deng W et al: Pulmonary MALT Lymphoma has variable features on CT. Sci Rep. 9(1):8657, 2019
                                            4. Yao D et al: Clinical and misdiagnosed analysis of primary pulmonary lymphoma: a retrospective study. BMC Cancer. 18(1):281, 2018
                                            5. Bligh MP et al: Spectrum of CT findings in thoracic extranodal non-Hodgkin lymphoma. Radiographics. 37(2):439-61, 2017
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Primary pulmonary lymphoma (PPL)
                                                          • Non-Hodgkin lymphoma (NHL)
                                                            • Mucosa-associated lymphoid tissue (MALT) lymphoma
                                                            • Diffuse large B-cell lymphoma (DLBCL)
                                                            • Lymphomatoid granulomatosis (LG)
                                                          • Hodgkin lymphoma (HL)
                                                        • Secondary pulmonary lymphoma (SPL)
                                                      • Synonyms

                                                        • Low-grade primary pulmonary B-cell lymphoma
                                                        • MALT lymphoma
                                                      • Definitions

                                                        • PPL
                                                          • Pulmonary clonal lymphoid proliferation in patient with no detectable extrapulmonary lymphoma for at least 3 months after initial diagnosis
                                                          • Represents 0.5% of primary lung malignancies
                                                          • Almost all cases of PPL are NHL
                                                            • ~ 70 cases of primary pulmonary HL reported
                                                          • Represents only 3-4% of cases of primary extranodal NHL
                                                          • Most common subtypes: MALT lymphoma, high-grade DLBCL, LG
                                                            • MALT lymphoma: Low-grade extranodal B-cell lymphoma, represents majority of cases of PPL
                                                          • Uncommon subtypes: Pulmonary intravascular lymphoma, primary pulmonary plasmacytoma
                                                        • SPL
                                                          • Common as manifestation of systemic involvement by HL and NHL
                                                          • Lung involvement rate higher in HL (85%) than in NHL (24%), but NHL is far more common and represents up to 90% of all SPL
                                                          • Mechanisms of lung involvement
                                                            • Invasion from hilar or mediastinal lymph nodes
                                                            • Hematogeneous or lymphatic dissemination

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • Imaging Recommendations

                                                              • Nuclear Medicine Findings

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Demographics

                                                                          • Mucosa-Associated Lymphoid Tissue Lymphoma

                                                                            • High-Grade B-Cell Non-Hodgkin Lymphoma

                                                                              • Lymphomatoid Granulomatosis

                                                                                • Primary Pulmonary Plasmacytoma

                                                                                  • Pulmonary Intravascular Lymphoma

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Consider

                                                                                      Selected References

                                                                                      1. Angirish B et al: Pulmonary manifestations of lymphoma: A pictorial essay. Lung India. 37(3):263-7, 2020
                                                                                      2. Cozzi D et al: Primary pulmonary lymphoma: imaging findings in 30 cases. Radiol Med. 124(12):1262-9, 2019
                                                                                      3. Deng W et al: Pulmonary MALT Lymphoma has variable features on CT. Sci Rep. 9(1):8657, 2019
                                                                                      4. Yao D et al: Clinical and misdiagnosed analysis of primary pulmonary lymphoma: a retrospective study. BMC Cancer. 18(1):281, 2018
                                                                                      5. Bligh MP et al: Spectrum of CT findings in thoracic extranodal non-Hodgkin lymphoma. Radiographics. 37(2):439-61, 2017