Encyclopedia of AIDS

Living Edition
| Editors: Thomas J. Hope, Douglas Richman, Mario Stevenson

AIDS-Related Primary Central Nervous System Lymphoma

  • Jan Davidson-Moncada
  • Thomas S. Uldrick
Living reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9610-6_16-1

Definition

Primary central nervous system lymphoma (PCNSL) is a rare extra-nodal B-cell non-Hodgkin lymphoma, which arises in the brain, spinal cord, meninges, or eyes. In individuals infected with HIV, PCNSL occurs with advanced immunosuppression and low CD4 T-cell count. It is considered an AIDS-defining malignancy. Unlike PCNSL in immune-competent hosts, AIDS-related PCNSL (AR-PCNSL) is almost exclusively caused by a cancer-causing herpesvirus, Epstein Barr Virus (EBV, also known as human herpesvirus-4). While PCNSL in immune-competent patients and AR-PCNSL have pathological and clinical overlap, AR-PCNSL is distinguished by its strong association with immunosuppression and viral etiology.

Introduction

Primary central nervous system lymphoma (PCNSL) is a rare extra-nodal non-Hodgkin lymphoma, which accounts for approximately 3–5 % of primary brain tumors. Prior to effective therapy for HIV, a large proportion of cases of PCNSL, especially in younger individuals, occurred in severely...

Keywords

Cerebral Spinal Fluid Primary Central Nervous System Lymphoma Leptomeningeal Dissemination Primary Central Nervous System Lymphoma Patient Systemic DLBCL 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Abrey LE, Batchelor TT, Ferreri AJ, Gospodarowicz M, Pulczynski EJ, Zucca E, et al. Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma. J Clin Oncol. 2005;23(22):5034–43.PubMedCrossRefGoogle Scholar
  2. Antinori A, De Rossi G, Ammassari A, Cingolani A, Murri R, Di Giuda D, 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(2):554–60.PubMedGoogle Scholar
  3. Balmaceda C, Gaynor JJ, Sun M, Gluck JT, DeAngelis LM. Leptomeningeal tumor in primary central nervous system lymphoma: recognition, significance, and implications. Ann Neurol. 1995;38(2):202–9.PubMedCrossRefGoogle Scholar
  4. Birnbaum T, Stadler EA, von Baumgarten L, Straube A. Rituximab significantly improves complete response rate in patients with primary CNS lymphoma. J Neurooncol. 2012;109(2):285–91.PubMedCrossRefGoogle Scholar
  5. Carbone A, Gaidano G, Gloghini A, Larocca LM, Capello D, Canzonieri V, et al. Differential expression of BCL-6, CD138/syndecan-1, and Epstein-Barr virus-encoded latent membrane protein-1 identifies distinct histogenetic subsets of acquired immunodeficiency syndrome-related non-Hodgkin’s lymphomas. Blood. 1998;91(3):747–55.PubMedGoogle Scholar
  6. Castellano-Sanchez AA, Li S, Qian J, Lagoo A, Weir E, Brat DJ. Primary central nervous system posttransplant lymphoproliferative disorders. Am J Clin Pathol. 2004;121(2):246–53.PubMedCrossRefGoogle Scholar
  7. Cherney BW, Sgadari C, Kanegane C, Wang F, Tosato G. Expression of the Epstein-Barr virus protein LMP1 mediates tumor regression in vivo. Blood. 1998;91(7):2491–500.PubMedGoogle Scholar
  8. Correa DD, DeAngelis LM, Shi W, Thaler H, Glass A, Abrey LE. Cognitive functions in survivors of primary central nervous system lymphoma. Neurology. 2004;62(4):548–55.PubMedCrossRefGoogle Scholar
  9. Ferreri AJ, Blay JY, Reni M, et al. Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience. J Clin Oncol. 2003;21(2):266–72.PubMedCrossRefGoogle Scholar
  10. Fischer L, Martus P, Weller M, et al. Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology. 2008;71(14):1102–8.PubMedCrossRefGoogle Scholar
  11. Gaidano G, Pasqualucci L, Capello D, Berra E, Deambrogi C, Rossi D, et al. Aberrant somatic hypermutation in multiple subtypes of AIDS-associated non-Hodgkin lymphoma. Blood. 2003;102(5):1833–41.PubMedCrossRefGoogle Scholar
  12. Gasser O, Bihl FK, Wolbers M, Loggi E, Steffen I, Hirsch HH, et al. HIV patients developing primary CNS lymphoma lack EBV-specific CD4+ T cell function irrespective of absolute CD4+ T cell counts. PLoS Med. 2007;4(3):e96.PubMedCentralPubMedCrossRefGoogle Scholar
  13. Heslop HE. How I treat EBV lymphoproliferation. Blood. 2009;114(19):4002–8.PubMedCentralPubMedCrossRefGoogle Scholar
  14. Hoffmann C, Tabrizian S, Wolf E, Eggers C, Stoehr A, Plettenberg A, et al. Survival of AIDS patients with primary central nervous system lymphoma is dramatically improved by HAART-induced immune recovery. AIDS. 2001;15(16):2119–27.PubMedCrossRefGoogle Scholar
  15. Jacomet C, Girard PM, Lebrette MG, Farese VL, Monfort L, Rozenbaum W. Intravenous methotrexate for primary central nervous system non-Hodgkin’s lymphoma in AIDS. AIDS. 1997;11(14):1725–30.PubMedCrossRefGoogle Scholar
  16. Kung CP, Raab-Traub N. Epstein-Barr virus latent membrane protein 1 modulates distinctive NF- kappaB pathways through C-terminus-activating region 1 to regulate epidermal growth factor receptor expression. J Virol. 2010;84(13):6605–14.PubMedCentralPubMedCrossRefGoogle Scholar
  17. MacMahon EM, Glass JD, Hayward SD, Mann RB, Becker PS, Charache P, et al. Epstein-Barr virus in AIDS-related primary central nervous system lymphoma. Lancet. 1991;338(8773):969–73.PubMedCrossRefGoogle Scholar
  18. Norden AD, Drappatz J, Wen PY, Claus EB. Survival among patients with primary central nervous system lymphoma, 1973–2004. J Neuro-Oncol. 2011;101(3):487–93.CrossRefGoogle Scholar
  19. Rubenstein JL, Fridlyand J, Shen A, Aldape K, Ginzinger D, Batchelor T, et al. Gene expression and angiotropism in primary CNS lymphoma. Blood. 2006;107(9):3716–23.PubMedCentralPubMedCrossRefGoogle Scholar
  20. Shiels MS, Pfeiffer RM, Hall HI, Li J, Goedert JJ, Morton LM, 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(14):1450–9.PubMedCentralPubMedCrossRefGoogle Scholar
  21. Tun HW, Personett D, Baskerville KA, et al. Pathway analysis of primary central nervous system lymphoma. Blood. 2008;111(6):3200–10.PubMedCentralPubMedCrossRefGoogle Scholar
  22. Wang L, Toomey NL, Diaz LA, Walker G, Ramos JC, Barber GN, et al. Oncogenic IRFs provide a survival advantage for Epstein-Barr virus- or human T-cell leukemia virus type 1-transformed cells through induction of BIC expression. J Virol. 2011;85(16):8328–37.PubMedCentralPubMedCrossRefGoogle Scholar
  23. Xu D, Zhao L, Del Valle L, Miklossy J, Zhang L. Interferon regulatory factor 4 is involved in Epstein-Barr virus-mediated transformation of human B lymphocytes. J Virol. 2008;82(13):6251–8.PubMedCentralPubMedCrossRefGoogle Scholar
  24. Ziegler JL, Beckstead JA, Volberding PA, Abrams DI, Levine AM, Lukes RJ, et al. Non-Hodgkin’s lymphoma in 90 homosexual men. Relation to generalized lymphadenopathy and the acquired immunodeficiency syndrome. N Engl J Med. 1984;311(9):565–70.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Medical Oncology BranchCenter for Cancer Research, National Cancer Institute (NCI)BethesdaUSA
  2. 2.Retroviral Disease Section, HIV and AIDS Malignancy BranchCenter for Cancer Research, National Cancer Institute (NCI)BethesdaUSA