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Orbital Lymphoproliferative Lesions
H. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Orbital lymphoproliferative lesions (OLPL)
              • Synonyms

                • Ocular adnexal lymphoproliferative lesions
                • Mucosa-associated lymphoid tissue (MALT) lymphoma = extranodal marginal zone lymphoma (ENMZL)
              • Definitions

                • OLPL represents spectrum of lesions ranging from benign lymphoid hyperplasia (LH) to malignant non-Hodgkin lymphoma (NHL)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Andrew NH et al: Lymphoid Hyperplasia of the Orbit and Ocular Adnexa: A Clinico-Pathological Review. Surv Ophthalmol. ePub, 2016
                                                2. Xu XQ et al: Benign and malignant orbital lymphoproliferative disorders: Differentiating using multiparametric MRI at 3.0T. J Magn Reson Imaging. ePub, 2016
                                                3. Amin S et al: Diagnostic pitfalls in "low-grade lymphoma" of the orbit and lacrimal gland. Orbit. 34(4):206-11, 2015
                                                4. Andrew NH et al: An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD. Br J Ophthalmol. 99(3):376-81, 2015
                                                5. Kharod SM et al: Radiotherapy in the management of orbital lymphoma: a single institution's experience over 4 decades. Am J Clin Oncol. ePub, 2015
                                                6. Haradome K et al: Orbital lymphoproliferative disorders (OLPDs): value of MR imaging for differentiating orbital lymphoma from benign OPLDs. AJNR Am J Neuroradiol. 35(10):1976-82, 2014
                                                7. Andrew NH et al: Intraorbital corticosteroid injection for orbital reactive lymphoid hyperplasia. Eye (Lond). 27(4):561-3, 2013
                                                8. Ho HH et al: Treatment of benign lymphoid hyperplasia of the orbit with rituximab. Ophthal Plast Reconstr Surg. 26(1):11-3, 2010
                                                9. Cohen VM: Treatment options for ocular adnexal lymphoma (OAL). Clin Ophthalmol. 3:689-92, 2009
                                                10. Sokol JA et al: Rituximab immunotherapy for ocular adnexal lymphoma: clinicopathologic correlation with 5-year follow-up. Ophthal Plast Reconstr Surg. 25(4):322-4, 2009
                                                11. Demirci H et al: Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 115(9):1626-31, 1631, 2008
                                                12. Dimitrakopoulos I et al: Effect of chemotherapy on primary mucosa-associated lymphoid tissue lymphoma of the orbit. J Oral Maxillofac Surg. 66(1):16-20, 2008
                                                13. Gayed I et al: Value of positron emission tomography in staging ocular adnexal lymphomas and evaluating their response to therapy. Ophthalmic Surg Lasers Imaging. 38(4):319-25, 2007
                                                14. Sullivan TJ et al: Imaging features of ocular adnexal lymphoproliferative disease. Eye (Lond). 20(10):1189-95, 2006
                                                15. Valenzuela AA et al: Positron emission tomography in the detection and staging of ocular adnexal lymphoproliferative disease. Ophthalmology. 113(12):2331-7, 2006
                                                16. Akansel G et al: MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol. 53(2):175-81, 2005
                                                17. Hasegawa M et al: Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification. Int J Radiat Oncol Biol Phys. 57(1):172-6, 2003
                                                18. Moon WJ et al: Orbital lymphoma and subacute or chronic inflammatory pseudotumor: differentiation with two-phase helical computed tomography. J Comput Assist Tomogr. 27(4):510-6, 2003
                                                19. Uno T et al: Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 98(4):865-71, 2003
                                                20. Douglas RS et al: Orbital presentation of posttransplantation lymphoproliferative disorder: a small case series. Ophthalmology. 109(12):2351-5, 2002
                                                21. Esmaeli B et al: Clinical presentation and treatment of secondary orbital lymphoma. Ophthal Plast Reconstr Surg. 18(4):247-53, 2002
                                                22. Le QT et al: Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys. 52(3):657-63, 2002
                                                23. Pelloski CE et al: Clinical stage IEA-IIEA orbital lymphomas: outcomes in the era of modern staging and treatment. Radiother Oncol. 59(2):145-51, 2001
                                                24. Shields CL et al: Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 108(5):979-84, 2001
                                                25. Valvassori GE et al: Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am. 37(1):135-50, x-xi, 1999
                                                26. Weber AL et al: Lymphoproliferative disease of the orbit. Neuroimaging Clin N Am. 6(1):93-111, 1996
                                                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

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Orbital lymphoproliferative lesions (OLPL)
                                                            • Synonyms

                                                              • Ocular adnexal lymphoproliferative lesions
                                                              • Mucosa-associated lymphoid tissue (MALT) lymphoma = extranodal marginal zone lymphoma (ENMZL)
                                                            • Definitions

                                                              • OLPL represents spectrum of lesions ranging from benign lymphoid hyperplasia (LH) to malignant non-Hodgkin lymphoma (NHL)

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Nuclear Medicine Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Andrew NH et al: Lymphoid Hyperplasia of the Orbit and Ocular Adnexa: A Clinico-Pathological Review. Surv Ophthalmol. ePub, 2016
                                                                                              2. Xu XQ et al: Benign and malignant orbital lymphoproliferative disorders: Differentiating using multiparametric MRI at 3.0T. J Magn Reson Imaging. ePub, 2016
                                                                                              3. Amin S et al: Diagnostic pitfalls in "low-grade lymphoma" of the orbit and lacrimal gland. Orbit. 34(4):206-11, 2015
                                                                                              4. Andrew NH et al: An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD. Br J Ophthalmol. 99(3):376-81, 2015
                                                                                              5. Kharod SM et al: Radiotherapy in the management of orbital lymphoma: a single institution's experience over 4 decades. Am J Clin Oncol. ePub, 2015
                                                                                              6. Haradome K et al: Orbital lymphoproliferative disorders (OLPDs): value of MR imaging for differentiating orbital lymphoma from benign OPLDs. AJNR Am J Neuroradiol. 35(10):1976-82, 2014
                                                                                              7. Andrew NH et al: Intraorbital corticosteroid injection for orbital reactive lymphoid hyperplasia. Eye (Lond). 27(4):561-3, 2013
                                                                                              8. Ho HH et al: Treatment of benign lymphoid hyperplasia of the orbit with rituximab. Ophthal Plast Reconstr Surg. 26(1):11-3, 2010
                                                                                              9. Cohen VM: Treatment options for ocular adnexal lymphoma (OAL). Clin Ophthalmol. 3:689-92, 2009
                                                                                              10. Sokol JA et al: Rituximab immunotherapy for ocular adnexal lymphoma: clinicopathologic correlation with 5-year follow-up. Ophthal Plast Reconstr Surg. 25(4):322-4, 2009
                                                                                              11. Demirci H et al: Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 115(9):1626-31, 1631, 2008
                                                                                              12. Dimitrakopoulos I et al: Effect of chemotherapy on primary mucosa-associated lymphoid tissue lymphoma of the orbit. J Oral Maxillofac Surg. 66(1):16-20, 2008
                                                                                              13. Gayed I et al: Value of positron emission tomography in staging ocular adnexal lymphomas and evaluating their response to therapy. Ophthalmic Surg Lasers Imaging. 38(4):319-25, 2007
                                                                                              14. Sullivan TJ et al: Imaging features of ocular adnexal lymphoproliferative disease. Eye (Lond). 20(10):1189-95, 2006
                                                                                              15. Valenzuela AA et al: Positron emission tomography in the detection and staging of ocular adnexal lymphoproliferative disease. Ophthalmology. 113(12):2331-7, 2006
                                                                                              16. Akansel G et al: MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol. 53(2):175-81, 2005
                                                                                              17. Hasegawa M et al: Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification. Int J Radiat Oncol Biol Phys. 57(1):172-6, 2003
                                                                                              18. Moon WJ et al: Orbital lymphoma and subacute or chronic inflammatory pseudotumor: differentiation with two-phase helical computed tomography. J Comput Assist Tomogr. 27(4):510-6, 2003
                                                                                              19. Uno T et al: Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 98(4):865-71, 2003
                                                                                              20. Douglas RS et al: Orbital presentation of posttransplantation lymphoproliferative disorder: a small case series. Ophthalmology. 109(12):2351-5, 2002
                                                                                              21. Esmaeli B et al: Clinical presentation and treatment of secondary orbital lymphoma. Ophthal Plast Reconstr Surg. 18(4):247-53, 2002
                                                                                              22. Le QT et al: Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys. 52(3):657-63, 2002
                                                                                              23. Pelloski CE et al: Clinical stage IEA-IIEA orbital lymphomas: outcomes in the era of modern staging and treatment. Radiother Oncol. 59(2):145-51, 2001
                                                                                              24. Shields CL et al: Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 108(5):979-84, 2001
                                                                                              25. Valvassori GE et al: Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am. 37(1):135-50, x-xi, 1999
                                                                                              26. Weber AL et al: Lymphoproliferative disease of the orbit. Neuroimaging Clin N Am. 6(1):93-111, 1996