Primary lymphomas of the central nervous system. Questions of epidemiology and treatment. Clinical example of multidisciplinary approach in therapy

Background. Currently, the frequency of occurrence of neoplasms is emerging in the world. Under conditions of a general tumor, brain tumors are rare (0.7–1.5 %). However, high mortality, disability, limited life expectancy of patients, high economic and social inferiority, raise concerns about the medical organization of care, development and social adaptation. Of particular importance among the causes of primary brain tumors is the congenital or acquired growth of immunodeficiency. The growth in the number of drug addicts led to the elimination of the annual epidemic at the end of the 70s and the increase in the number of patients with immunodeficiency syndrome (AIDS), due to which the growth of a brain tumor began to develop in this population group. The number of Russian patients with lymphoma is also steadily increasing.

Objective. Consider an illustrative clinical case that reflects the expected continuity in patients with ordinary CNS lymphoma.

Design and methods. A 50-year-old patient with moderate memory loss was examined using various methods: MRI, PET-CT and etc.

Results. Based on histological and immunohistochemical studies, diffuse large B-cell lymphoma of the CNS was diagnosed. There was a positive trend in the reduction of tumor size after radiation therapy.

Conclusion. Primary CNS lymphoma is becoming an active epidemiological problem of society, requiring comprehensive multidisciplinary monitoring. Only high vigilance in terms of lymphoma and well-coordinated work of multidisciplinary specialists can help to perform the correct diagnostics and patient management.

1. Komratova KA, Abugova YG, Ozerov SS, et al. Primary diffuse large B-cell lymphoma of the central nervous system (case report and literature review). Oncohematology. 2017 Jan 1;12(1):10–6. Available from: http://oncohematology.abvpress.ru/ongm/article/view/227

2. Quinn T Ostrom, Gino C, Kristin W, et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014–2018, Neuro-Oncology, Volume 23, Issue Supplement_3, October 2021, Pages iii1–iii105, https://doi.org/10.1093/neuonc/noab200

3. Eby NL, Grufferman S, Flannelly CM, et al. Increasing incidence of primary brain lymphoma in the US. Cancer. 1988 Dec 1;62(11):2461–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/3179963

4. Bailey P. Intracranial sarcomatous tumors of leptomeningeal origin. Arch Surg. 1929;18:1359–402.

5. Henry JM, Heffner RR, Dillard SH, et al. Primary malignant lymphomas of the central nervous system. Cancer. 1974 Oct;34(4):1293–302. Available from: https://onlinelibrary.wiley.com/doi/10.1002/1097-0142(197410)34:4%3C1293::AID-CNCR2820340441%3E3.0.CO;2-P

6. Esptein LG, DiCarlo FJ, Joshi V V, et al. Primary lymphoma of the central nervous system in children with acquired immunodeficiency syndrome. Pediatrics. 1988 Sep;82(3):355–63. Available from: http://www.ncbi.nlm.nih.gov/pubmed/3261418

7. Itoyama T, Sadamori N, Tsutsumi K, et al. Primary central nervous system lymphomas. Immunophenotypic, virologic, and cytogenetic findings of three patients without immune defects. Cancer. 1994 Jan 15;73(2):455–63. Available from: https://onlinelibrary.wiley.com/doi/10.1002/1097-0142(19940115)73:2%3C455::AID-CNCR2820730234%3E3.0.CO;2-0

8. Zhang L, Zhang J, Lambert Q, et al. Interferon regulatory factor 7 is associated with Epstein-Barr virus-transformed central nervous system lymphoma and has oncogenic properties. J Virol. 2004 Dec;78(23):12987–95. Available from: http://www.ncbi.nlm.nih.gov/pubmed/15542650

9. Del Valle L, Enam S, Lara C, et al. Primary Central Nervous System Lymphoma Expressing the Human Neurotropic Polyomavirus, JC Virus, Genome. J Virol. 2004 Apr;78(7):3462–9. Available from: https://journals.asm.org/doi/10.1128/JVI.78.7.3462-3469.2004

10. Shenkier T, Connors J. Primary extranodal non-Hodgkin’s lymphomas. The lymphomas, 2nd edn. Philadelphia: Saunders Elsevier; 2006. 325–347 p.

11. Mohile NA, Abrey LE. Primary Central Nervous System Lymphoma. Neurol Clin. 2007 Nov;25(4):1193–207. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0733861907000734

12. Gliemroth J, Kehler U, Gaebel C, et al. Neuroradiological findings in primary cerebral lymphomas of non-AIDS patients. Clin Neurol Neurosurg. 2003 Apr;105(2):78–86. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0303846702001051

13. Hayakawa T, Takakura K, Abe H, et al. Primary central nervous system lymphoma in Japan--a retrospective, co-operative study by CNS-Lymphoma Study Group in Japan. J Neurooncol. 1994;19(3):197–215. Available from: http://link.springer.com/10.1007/BF01053274

14. Bataille B, Delwail V, Menet E, et al. Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 2000 Feb;92(2):261–6. Available from: https://thejns.org/view/journals/j-neurosurg/92/2/article-p261.xml

15. Bühring U, Herrlinger U, Krings T, et al. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001 Aug 14;57(3):393–6. Available from: http://www.neurology.org/lookup/doi/10.1212/WNL.57.3.393

16. Haldorsen IS, Krossnes BK, Aarseth JH, et al. Increasing incidence and continued dismal outcome of primary central nervous system lymphoma in Norway 1989-2003 : time trends in a 15-year national survey. Cancer. 2007 Oct 15;110(8):1803–14. Available from: https://onlinelibrary.wiley.com/doi/10.1002/cncr.22989

17. Olson JE, Janney CA, Rao RD, et al. The continuing increase in the incidence of primary central nervous system non-Hodgkin lymphoma: a surveillance, epidemiology, and end results analysis. Cancer. 2002 Oct 1;95(7):1504–10. Available from: https://onlinelibrary.wiley.com/doi/10.1002/cncr.10851

18. Poddubnaya IV, Savchenko VG. Russian clinical guidelines for the diagnosis and treatment of lymphoproliferative diseases: standards of patient management for physicians. Moscow: Buki Wedi, 2018. P. 470. In Russian.

19. Ferreri A. J. M. 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 //The Lancet. – 2009. – Т. 374. – №. 9700. – С. 1512-1520

20. Kobyakov GL, Bekyashev AH, Golanov AV, et al. Practical recommendations for drug treatment of primary tumors of the Central nervous system. Malignant tumors: RUSSCO practical recommendation. 2018;8:83–99. In Russian.

21. Bashir R, Freedman A, Harris N, et al. Immunophenotypic profile of CNS lymphoma: a review of eighteen cases. J Neurooncol. 1989 Sep;7(3):249–54. Available from: http://link.springer.com/10.1007/BF00172918