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Diagnosis
Mediastinal Abnormalities
Lymphadenopathy
Primary Mediastinal Lymphoma
Primary Mediastinal Lymphoma
Santiago Martínez-Jiménez, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Primary mediastinal lymphoma (PML)

            • Definitions

              • Primary lymphoma: Lymphoma primarily localized to region ± little systemic disease (e.g., primary CNS, primary breast, primary mediastinal non-Hodgkin lymphoma [NHL])
              • PML: Lymphoma arising from thymus, mainly or exclusively involving anterior mediastinum; small disease burden may be present in other mediastinal compartments or outside mediastinum including other intra- or extrathoracic organs
                • Mediastinal diffuse large B-cell lymphoma (DLBCL): Common mediastinal neoplasm arising in thymic B cells from either germinal center or nongerminal center
                • Hodgkin lymphoma (HL)
                  • Most common presenting manifestation is cervical lymphadenopathy (LAD); 2nd most common presenting manifestation is large anterior mediastinal mass
                  • When HL predominantly affects anterior mediastinum, some physicians consider it PML
                    • Clinically and radiologically indistinguishable from other primary mediastinal NHL
                • Mediastinal gray-zone lymphoma (GZL): Rare PML with features intermediate between mediastinal DLBCL and HL (nodular sclerosis)
                  • Also known as B-cell lymphoma; unclassifiable
                  • Was considered within HL-like anaplastic large-cell lymphoma before its definition
                • Mediastinal T-cell lymphoblastic lymphoma (TLBL): Rare primary mediastinal neoplasm of precursor T-cell origin that affects young individuals

            IMAGING

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • Staging, Grading, & Classification

                        • Histology

                          • Molecular Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Dunleavy K et al: Emerging biological insights and novel treatment strategies in primary mediastinal large B-cell lymphoma. Semin Hematol. 52(2):119-125, 2015
                                    2. Evens AM et al: Gray zone lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma: characteristics, outcomes, and prognostication among a large multicenter cohort. Am J Hematol. ePub, 2015
                                    3. Littooij AS et al: Whole-body MRI-DWI for assessment of residual disease after completion of therapy in lymphoma: A prospective multicenter study. J Magn Reson Imaging. ePub, 2015
                                    4. Moskowitz CH et al: Current status of the role of PET imaging in diffuse large B-cell lymphoma. Semin Hematol. 52(2):138-142, 2015
                                    5. Xie Y et al: The histological classification of diffuse large B-cell lymphomas. Semin Hematol. 52(2):57-66, 2015
                                    6. Barrington SF et al: Role of imaging in the staging and response assessment of lymphoma: consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol. 32(27):3048-58, 2014
                                    7. Cheson BD et al: Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 32(27):3059-68, 2014
                                    8. Dabrowska-Iwanicka A et al: Primary mediastinal large B-cell lymphoma. Curr Hematol Malig Rep. 9(3):273-83, 2014
                                    9. Ellin F et al: Treatment outcome in T-cell lymphoblastic lymphoma in adults - a population-based study from the Swedish Lymphoma Registry. Acta Oncol. 53(7):927-34, 2014
                                    10. Wilson WH et al: A prospective study of mediastinal gray-zone lymphoma. Blood. 124(10):1563-9, 2014
                                    11. Grant C et al: Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: what is the oncologist to do? Curr Hematol Malig Rep. 6(3):157-63, 2011
                                    12. Lin C et al: Whole-body diffusion-weighted imaging in lymphoma. Cancer Imaging. 10 Spec no A:S172-8, 2010
                                    13. Johnson PW et al: Primary mediastinal B-cell lymphoma. Hematology Am Soc Hematol Educ Program. 2008:349-58, 2008
                                    14. Traverse-Glehen A et al: Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. Am J Surg Pathol. 29(11):1411-21, 2005
                                    15. Tateishi U et al: Primary mediastinal lymphoma: characteristic features of the various histological subtypes on CT. J Comput Assist Tomogr. 28(6):782-9, 2004
                                    16. Willemssen F et al: Mediastinal T-cell lymphoblastic lymphoma. JBR-BTR. 85(3):172-3, 2002
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Primary mediastinal lymphoma (PML)

                                              • Definitions

                                                • Primary lymphoma: Lymphoma primarily localized to region ± little systemic disease (e.g., primary CNS, primary breast, primary mediastinal non-Hodgkin lymphoma [NHL])
                                                • PML: Lymphoma arising from thymus, mainly or exclusively involving anterior mediastinum; small disease burden may be present in other mediastinal compartments or outside mediastinum including other intra- or extrathoracic organs
                                                  • Mediastinal diffuse large B-cell lymphoma (DLBCL): Common mediastinal neoplasm arising in thymic B cells from either germinal center or nongerminal center
                                                  • Hodgkin lymphoma (HL)
                                                    • Most common presenting manifestation is cervical lymphadenopathy (LAD); 2nd most common presenting manifestation is large anterior mediastinal mass
                                                    • When HL predominantly affects anterior mediastinum, some physicians consider it PML
                                                      • Clinically and radiologically indistinguishable from other primary mediastinal NHL
                                                  • Mediastinal gray-zone lymphoma (GZL): Rare PML with features intermediate between mediastinal DLBCL and HL (nodular sclerosis)
                                                    • Also known as B-cell lymphoma; unclassifiable
                                                    • Was considered within HL-like anaplastic large-cell lymphoma before its definition
                                                  • Mediastinal T-cell lymphoblastic lymphoma (TLBL): Rare primary mediastinal neoplasm of precursor T-cell origin that affects young individuals

                                              IMAGING

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Nuclear Medicine Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • Staging, Grading, & Classification

                                                          • Histology

                                                            • Molecular Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Dunleavy K et al: Emerging biological insights and novel treatment strategies in primary mediastinal large B-cell lymphoma. Semin Hematol. 52(2):119-125, 2015
                                                                      2. Evens AM et al: Gray zone lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma: characteristics, outcomes, and prognostication among a large multicenter cohort. Am J Hematol. ePub, 2015
                                                                      3. Littooij AS et al: Whole-body MRI-DWI for assessment of residual disease after completion of therapy in lymphoma: A prospective multicenter study. J Magn Reson Imaging. ePub, 2015
                                                                      4. Moskowitz CH et al: Current status of the role of PET imaging in diffuse large B-cell lymphoma. Semin Hematol. 52(2):138-142, 2015
                                                                      5. Xie Y et al: The histological classification of diffuse large B-cell lymphomas. Semin Hematol. 52(2):57-66, 2015
                                                                      6. Barrington SF et al: Role of imaging in the staging and response assessment of lymphoma: consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol. 32(27):3048-58, 2014
                                                                      7. Cheson BD et al: Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 32(27):3059-68, 2014
                                                                      8. Dabrowska-Iwanicka A et al: Primary mediastinal large B-cell lymphoma. Curr Hematol Malig Rep. 9(3):273-83, 2014
                                                                      9. Ellin F et al: Treatment outcome in T-cell lymphoblastic lymphoma in adults - a population-based study from the Swedish Lymphoma Registry. Acta Oncol. 53(7):927-34, 2014
                                                                      10. Wilson WH et al: A prospective study of mediastinal gray-zone lymphoma. Blood. 124(10):1563-9, 2014
                                                                      11. Grant C et al: Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: what is the oncologist to do? Curr Hematol Malig Rep. 6(3):157-63, 2011
                                                                      12. Lin C et al: Whole-body diffusion-weighted imaging in lymphoma. Cancer Imaging. 10 Spec no A:S172-8, 2010
                                                                      13. Johnson PW et al: Primary mediastinal B-cell lymphoma. Hematology Am Soc Hematol Educ Program. 2008:349-58, 2008
                                                                      14. Traverse-Glehen A et al: Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. Am J Surg Pathol. 29(11):1411-21, 2005
                                                                      15. Tateishi U et al: Primary mediastinal lymphoma: characteristic features of the various histological subtypes on CT. J Comput Assist Tomogr. 28(6):782-9, 2004
                                                                      16. Willemssen F et al: Mediastinal T-cell lymphoblastic lymphoma. JBR-BTR. 85(3):172-3, 2002