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Primary Central Nervous System Lymphoma: Neuro-Oncologic Approach

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Ocular and Adnexal Lymphoma

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

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Abstract

Primary central nervous system lymphoma (PCNSL) is a rare, aggressive, non-Hodgkin diffuse large B-cell lymphoma (DLBCL) in origin. It affects the central nervous system (CNS) compartments including the brain, cranial nerves, spinal cord, and meninges. Brain MRI with and without contrast is considered the method of choice to diagnose PCNSL. Treatment of PCNSL is controversial as it is a relatively rare malignancy with a heterogeneous patient population making comparative prospective trials more challenging. Most data are derived from retrospective, small prospective, and a few trials with variable results that will be summarized in this chapter.

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  • 25 June 2023

    A correction has been published.

References

  1. Jahnke K, et al. Relapse of primary central nervous system lymphoma: clinical features, outcome and prognostic factors. J Neuro-Oncol. 2006;80:159–65.

    Article  Google Scholar 

  2. Phillips EH, Fox CP, Cwynarski K. Primary CNS lymphoma. Curr Hematol Malig Rep. 2014;9:243–53.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Bataille B, et al. Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 2000;92:261–6.

    Article  CAS  PubMed  Google Scholar 

  4. Fine HA, Mayer RJ. Primary central nervous system lymphoma. Ann Intern Med. 1993;119:1093–104.

    Article  CAS  PubMed  Google Scholar 

  5. Küker W, et al. Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients. J Neuro-Oncol. 2005;72:169–77.

    Article  Google Scholar 

  6. Abrey LE, et al. Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma. J Clin Oncol. 2005;23:5034–43.

    Article  PubMed  Google Scholar 

  7. Hall KH, Panjic EH, Valla K, Flowers CR, Cohen JB. How to decide which DLBCL patients should receive CNS prophylaxis. Oncology. 2018;32:303–9.

    PubMed  Google Scholar 

  8. Porter AB, et al. Primary central nervous system lymphoma can be histologically diagnosed after previous corticosteroid use: a pilot study to determine whether corticosteroids prevent the diagnosis of primary central nervous system lymphoma. Ann Neurol. 2008;63:662–7.

    Article  CAS  PubMed  Google Scholar 

  9. Abrey LE, et al. Primary central nervous system lymphoma: the memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 2006;24:5711–5.

    Article  PubMed  Google Scholar 

  10. Ferreri AJM, et al. Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience. J Clin Oncol. 2003;21:266–72.

    Article  PubMed  Google Scholar 

  11. Kassem AN, Peereboom DM. Approach to the patient with CNS lymphoma. Neuro-oncology for the clinical neurologist. Amsterdam: Elsevier; 2021. p. 172–85. https://doi.org/10.1016/B978-0-323-69494-0.00013-0.

    Book  Google Scholar 

  12. Kerbauy MN, et al. Challenges and opportunities in primary CNS lymphoma: a systematic review. Radiother Oncol. 2017;122:352–61.

    Article  PubMed  Google Scholar 

  13. Grommes C, DeAngelis LM. Primary CNS lymphoma. J Clin Oncol. 2017;35:2410–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Shibamoto Y, et al. Results of radiation monotherapy for primary central nervous system lymphoma in the 1990s. Int J Radiat Oncol. 2005;62:809–13.

    Article  Google Scholar 

  15. Nelson DF, et al. Non-Hodgkin’s lymphoma of the brain: can high dose, large volume radiation therapy improve survival? Report on a prospective trial by the Radiation therapy Oncology Group (RTOG): RTOG 8315. Int J Radiat Oncol. 1992;23:9–17.

    Article  CAS  Google Scholar 

  16. Citterio G, María Ferreri AJ, Reni M. Current uses of radiation therapy in patients with primary CNS lymphoma. Expert Rev Anticancer Ther. 2014;13:1327–37. https://doi.org/10.1586/14737140.2013.851007.

    Article  CAS  Google Scholar 

  17. Alvarez-Pinzon AM, et al. Gamma knife stereotactic radiosurgery as an effective tool in primary CNS lymphoma: evaluation of stereotactic radiosurgery and methotrexate treatment in a prospective and observational clinical research study. Clin Neurol Neurosurg. 2021;201:106457.

    Article  PubMed  Google Scholar 

  18. Graham MS, DeAngelis LM. Improving outcomes in primary CNS lymphoma. Best Pract Res Clin Haematol. 2018;31:262–9.

    Article  PubMed  Google Scholar 

  19. Ferreri AJ, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet. 2009;374:1512–20.

    Article  CAS  PubMed  Google Scholar 

  20. Bromberg JEC, van der Meulen M, Doorduijn JK. The role of rituximab in primary central nervous system lymphoma. Curr Oncol Rep. 2020;22:78.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Angelov L, et al. Blood-brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed primary CNS lymphoma: a multi-institutional experience. J Clin Oncol. 2009;27:3503–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Korfel A, Thiel E, Martus P, Weller M. Whole-brain radiotherapy in primary CNS lymphoma – authors’ reply. Lancet Oncol. 2011;12:119–20.

    Article  Google Scholar 

  23. Citterio G, Reni M, Gatta G, Ferreri AJM. Primary central nervous system lymphoma. Crit Rev Oncol Hematol. 2017;113:97–110.

    Article  PubMed  Google Scholar 

  24. Omuro AMP, et al. Randomized phase II study of rituximab, methotrexate (MTX), procarbazine, vincristine, and cytarabine (R-MPV-A) with and without low-dose whole-brain radiotherapy (LD-WBRT) for newly diagnosed primary CNS lymphoma (PCNSL). J Clin Oncol. 2020;38:2501. https://doi.org/10.1200/JCO.2020.38.15_suppl.2501.

    Article  Google Scholar 

  25. Rubenstein JL, et al. Intensive chemotherapy and immunotherapy in patients with newly diagnosed primary CNS lymphoma: CALGB 50202 (Alliance 50202). J Clin Oncol. 2013;31:3061–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Bessell EM, Dickinson P, Dickinson S, Salmon J. Increasing age at diagnosis and worsening renal function in patients with primary central nervous system lymphoma. J Neuro-Oncol. 2011;104:191–3.

    Article  Google Scholar 

  27. Neuwelt EA, et al. Primary CNS lymphoma treated with osmotic blood-brain barrier disruption: prolonged survival and preservation of cognitive function. J Clin Oncol. 1991;9:1580–90.

    Article  CAS  PubMed  Google Scholar 

  28. Rios A. HIV-related hematological malignancies: a concise review. Clin Lymphoma Myeloma Leuk. 2014;14:S96–S103.

    Article  PubMed  Google Scholar 

  29. Forsyth PA, DeAngelis LM. Biology and management of AIDS-associated primary CNS lymphomas. Hematol Oncol Clin North Am. 1996;10:1125–34.

    Article  CAS  PubMed  Google Scholar 

  30. Shiels MS, et al. Proportions of Kaposi sarcoma, selected non-Hodgkin lymphomas, and cervical cancer in the United States occurring in persons with AIDS, 1980-2007. JAMA. 2011;305:1450.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Achenbach CJ, et al. Mortality after cancer diagnosis in HIV-infected individuals treated with antiretroviral therapy. AIDS. 2011;25:691–700.

    Article  PubMed  Google Scholar 

  32. Gupta NK, et al. Long-term survival in AIDS-related primary central nervous system lymphoma. Neuro-Oncology. 2017;19:99–108.

    Article  CAS  PubMed  Google Scholar 

  33. Antinori A, et al. Value of combined approach with thallium-201 single-photon emission computed tomography and Epstein-Barr virus DNA polymerase chain reaction in CSF for the diagnosis of AIDS-related primary CNS lymphoma. J Clin Oncol. 1999;17:554.

    Article  CAS  PubMed  Google Scholar 

  34. Kaplan LD, et al. Rituximab does not improve clinical outcome in a randomized phase 3 trial of CHOP with or without rituximab in patients with HIV-associated non-Hodgkin lymphoma: AIDS-Malignancies Consortium Trial 010. Blood. 2005;106:1538.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Evens AM, et al. Primary CNS posttransplant lymphoproliferative disease (PTLD): an international report of 84 cases in the modern era. Am J Transplant. 2013;13:1512–22.

    Article  CAS  PubMed  Google Scholar 

  36. Morgans AK, Reshef R, Tsai DE. Posttransplant lymphoproliferative disorder following kidney transplant. Am J Kidney Dis. 2010;55:168–80.

    Article  PubMed  Google Scholar 

  37. Mahale P, Shiels MS, Lynch CF, Engels EA. Incidence and outcomes of primary central nervous system lymphoma in solid organ transplant recipients. Am J Transplant. 2018;18:453–61.

    Article  CAS  PubMed  Google Scholar 

  38. Langner-Lemercier S, et al. Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network. Neuro-Oncology. 2016;18:1297–303.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Korfel A, et al. Randomized phase III study of whole-brain radiotherapy for primary CNS lymphoma. Neurology. 2015;84:1242–8.

    Article  CAS  PubMed  Google Scholar 

  40. Shenkier TN, et al. Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS Lymphoma Collaborative Group. J Clin Oncol. 2005;23:2233–9.

    Article  PubMed  Google Scholar 

  41. Siddiqi T, et al. CD19-directed CAR T-cell therapy for treatment of primary CNS lymphoma. Blood Adv. 2021;5:4059–63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Palmer JD, et al. Outcomes after stereotactic radiosurgery for CNS lymphoma. J Neuro Oncol. 2020;147(2):465–76.

    Article  Google Scholar 

  43. Korfel A, et al. Prognostic impact of meningeal dissemination in primary CNS lymphoma (PCNSL): experience from the G-PCNSL-SG1 trial. Ann Oncol. 2012;23:2374–80.

    Article  CAS  PubMed  Google Scholar 

  44. Taylor JW, et al. Primary leptomeningeal lymphoma: International Primary CNS Lymphoma Collaborative Group report. Neurology. 2013;81:1690.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Lachance DH, et al. Primary leptomeningeal lymphoma: report of 9 cases, diagnosis with immunocytochemical analysis, and review of the literature. Neurology. 1991;41:95–100.

    Article  CAS  PubMed  Google Scholar 

  46. Ferreri AJM, et al. Whole-brain radiotherapy in primary CNS lymphoma. Lancet Oncol. 2011;12:118–9.

    Article  PubMed  Google Scholar 

  47. Schaff LR, Grommes C. Updates on primary central nervous system lymphoma. Curr Oncol Rep. 2018;20:11.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Carson KR, et al. Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients: a report of 57 cases from the Research on Adverse Drug Events and Reports project. Blood. 2009;113:4834.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Correspondence to David M. Peereboom .

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Kassem, A.N., Peereboom, D.M. (2023). Primary Central Nervous System Lymphoma: Neuro-Oncologic Approach. In: Raval, V.R., Mruthyunjaya, P., Singh, A.D. (eds) Ocular and Adnexal Lymphoma. Essentials in Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-031-24595-4_11

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  • DOI: https://doi.org/10.1007/978-3-031-24595-4_11

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  • Print ISBN: 978-3-031-24594-7

  • Online ISBN: 978-3-031-24595-4

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