Primary central nervous system Burkitt lymphoma: Case report and literature review
V. A. Balayants,
D. A. Koroleva,
A. M. Kovrigina,
E. E. Zvonkov,
E. O. Kiselev,
T. N. Obukhova,
G. A. Yatsyk https://doi.org/10.35754/0234-5730-2025-70-4-530-541
Abstract
Introduction. Burkitt lymphoma is an aggressive B-cell lymphoma characterized by pathomorphological, immunophenotypic, and cytogenetic features that are the main criteria for its diagnosis. Burkitt lymphoma with isolated involvement of the central nervous system (CNS) is an extremely rare nosological form of primary lymphoma of the central nervous system. The small number of observations in the literature does not allow us to fully determine the main clinical, radiological and age-related features of this lymphoma, as well as standardize the treatment tactics for patients with primary Burkitt lymphoma of the central nervous system.
Aim: to present a clinical case of a patient with primary Burkitt lymphoma of the CNS with damage to the right hemisphere of the cerebellum and brainstem with compression of the cavity of the fourth ventricle.
Main findings. The diagnosis was established based on the basis of pathomorphological and immunohistochemical examination of the removed neoplasm of the fourth ventricle and was confirmed by in situ fluorescence hybridization using the DNA probe MYC gene break apart detection dual-color probe, which revealed a classical translocation involving the MYC/8q24 gene. The patient underwent 4 courses of chemotherapy under the LB-M-04 program, at the end of which a regression of the neoplasm was achieved.
About the Authors
V. A. Balayants
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Viktor A. Balayants, Pathologist, Pathology Department
125167, Moscow
D. A. Koroleva
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Daria A. Koroleva, Cand. Sci. (Med.), Hematologist, Department of Hematology and Chemotherapy of Lymphomas with Bone Marrow and Hematopoietic Stem Cell Transplantation Unit
125167, Moscow
A. M. Kovrigina
National Medical Research Center for Hematology; IM Sechenov First Moscow State Medical University
Russian Federation
Russian Federation
Alla M. Kovrigina, Dr. Sci. (Biol.), Head of the Pathology Department; Professor of the Department of the First Moscow State Medical University named
125167, Moscow
119991, Moscow
E. E. Zvonkov
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Evgeny E. Zvonkov, Dr. Sci. (Med), Head of the Department of Hematology and Chemotherapy of Lymphomas with Bone Marrow and Hematopoietic Stem Cell Transplantation Unit
125167, Moscow
E. O. Kiselev
Moscow Botkin Multidisciplinary Scientific-Clinical Center, Department of Health of the City of Moscow
Russian Federation
Russian Federation
Egor O. Kiselev, neurosurgeon, Neurosurgical Department No. 19
125284, Moscow
T. N. Obukhova
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Tatyana N. Obukhova, Cand. Sci. (Med.), Head of the Karyology Laboratory
125167, Moscow
G. A. Yatsyk
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Galina A. Yatsyk, Cand. Sci. (Med.), Head of the Magnetic Resonance Imaging Department
125167, Moscow
References
1. Swerdlow S.H., Campo E., Harris N.L., et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th ed. Lyon, France: International Agency for Research in Cancer (IARC), 2017. 585 с.
2. Ishaque N., Koll R., Gu Z., et al. The genomic and transcriptional landscape of primary central nervous system lymphoma. Nat Commun. 2022;13(2558):1–20. DOI: 10.1038/s41467-022-30050-y.
3. Alaggio R., Amador C., Anagnostopoulos I., et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36:1720–48. DOI: 10.1038/s41375-022-01620-2.
4. Koning M.E. De, Hof J.J., Jansen C., et al. Primary central nervous system lymphoma. J Neurol. 2023; 271(5):2906–13. DOI: 10.1007/s00415-023-12143-w.
5. Ahmed F., Nicolás K., Vishakha C., et al. Rare central nervous system lymphomas. Br J Haematol. 2022;197:662–78. DOI: 10.1111/bjh.18128.
6. Valsamis M.P., Levine P.H., Rapin I., et al. Primary intracranial Burkitt’s lymphoma in an infant. Cancer. 1976;37:1500–7. DOI: 10.1002/1097-0142(197603)37:33.0.co;2-w.
7. Gawish H.H.A. Primary Burkitt’s lymphoma of the frontal bone. J Neurosurg. 1976;45:712–5. DOI: 10.3171/jns.1976.45.6.0712.
8. Giromini D., Peiffer J., Tzonos T. Occurrence of a Primary Burkitt-Type Lymphoma of the Central Nervous System in an Astrocytoma Patient. Acta Neuropathol. 1981;54:165–7. DOI: 10.1007/BF00689412.
9. Hegediis K. Burkitt-Type Lymphoma and Reticulum-Cell Sarcoma An Unusual Mixed Form of Two Intracranial Primary Malignant Lymphomas. Surg Neurol. 1984;21:23–9. DOI: 10.1016/0090-3019(84)90395-1.
10. Kobayashi H., Sano T., Ii K., et al. Primary Burkitt-type Lymphoma of the Central Nervous System. Acta Neuropathol. 1984;64:12–4. DOI: 10.1007/BF00695600.
11. Pui C., Dahl G. V. Epidural spinal cord compression as the initial finding in childhood acute leukemia and non-Hodgkin lymphoma. Clin Lab Obs. 1985;106(5):788–92. DOI: 10.1016/S0022-3476(85)80357-7.
12. Mizugami T., Mikata A., Hajikano H., et al. Primary Spinal Epidural Burkitt’s Lymphoma. Surg Neurol. 1987;28:158–62. DOI: 10.1016/0090-3019(87)90092-9.
13. Shigemori M., Tokunaga T., Miyagi J., et al. Multiple Brain Tumors of Different Cell Types with an Unruptured Cerebral Aneurysm. Neurol Med Chir. 1989;31:96–9. DOI: 10.2176/nmc.31.96.
14. Tekkok I.H., Tahta K., Erbengi A., et al. Primary intracranial extradural Burkitttype lymphoma. Child’s Nerv Syst. 1991;7:172–4. DOI: 10.1007/BF00776718.
15. Toren A., Mandel M., Shahar E., et al. Primary Central Nervous System Burkitt’s lymphoma Presenting as Guillain-Barre Syndrome. Med Pediatr Oncol. 1994;23:372–5. DOI: 10.1002/mpo.2950230410.
16. Späth-Schwalbe E., Genvresse I., Stein H., et al. Primary cerebral highly-malignant B-cell lymphoma of the Burkitt type. Dtsch Med Wochenschr. 1999;124:451–5. DOI: 10.1002/mpo.2950230410.
17. Mora J. Primary Epidural Non-Hodgkin Lymphoma: Spinal Cord Compression Syndrome as the Initial Form of Presentation in Childhood Non-Hodgkin Lymphoma. Med Pediatr Oncol. 1999;32:102–5. DOI: 10.1002/(SICI)1096-911X(199902)32:2<102::AID-MPO6>3.0.CO;2-Y.
18. Wilkening A., Brack M., Brandis A., et al. Unusual presentation of a primary spinal Burkitt’s lymphoma. J Neurol Neurosurg Psychiatry. 2001;70:794–7. DOI: 10.1136/jnnp.70.6.794.
19. Monabati A.H.M., Akei S.M.O.R. Primary Burkitt lymphoma of the brain in an immunocompetent patient. J Neurosurg. 2002;96:1127–9. DOI: 10.3171/jns.2002.96.6.1127.
20. Daley M.F., Partington M.D., Kadan-lottick N., et al. Primary Epidural Burkitt Lymphoma in a Child: Case Presentation and Literature Review. Pediatr Hematol Oncol. 2016;20(4):333–8. DOI: 10.1080/08880010390203062.
21. Shehu B.B. Primary central nervous system Burkitt ’ s lymphoma presenting with proptosis. Ann ofTropical Paediatr. 2003;23:319–20. DOI: 10.1179/027249303322705812.
22. Huisman T.A.G.M., Tschirch F., Schneider J.F.L., et al. Burkitt’s lymphoma with bilateral cavernous sinus and mediastinal involvement in a child. Pediatr Radiol. 2003;33:719–21. DOI: 10.1007/s00247-003-1010-x.
23. Abel T.W., Thompson M.A., Kim J., et al. Primary central nervous system Burkitt lymphoma: report of a case confirmed with identification of t(8;14) by FISH. Brain Pathol. 2025;16(Supplement 1):96–7.
24. Gobbato P.L., Azambuja A. De, Filho P., et al. Primary meningeal Burkitt-type lymphoma peresenting as the first clinical manifestation of acquired immunodeficiency syndrome. Arq Neuropsiquiatr. 2006;64(2):511–5. DOI: 10.1590/S0004-282X2006000300030.
25. Kozáková D., Macháleková K., Brtko P., et al. Primary B-cell pituitary lymphoma of the Burkitt type: case report of the rare clinic entity with typical clinical presentation. Cas Lek Ces. 2008;147(11):569–73.
26. Gu Y., Zhang Y.H.X., Hu F. Primary central nervous system Burkitt lymphoma as concomitant lesions in the third and the left ventricles : a case study and literature review. J Neurooncol. 2010;99:277–81. DOI: 10.1007/s11060-010-0122-z.
27. Takasu M., Takeshita S., Tanitame N., et al. Primary hypothalamic third ventriclular Burkitt ’ s lymphoma: a case report with emphasis on differential diagnosis. Br J Radiol. 2010;83:43–7. DOI: 10.1259/bjr/84426981.
28. Jiang M., Zhu J., Guan Y., et al. Primary Central Nervous System Burkitt Lymphoma With Non-Immunoglobulin Heavy Chain Translocation in Right Ventricle: Case Report. Pediatr Hematol Oncol. 2011;28:454–8. DOI: 10.3109/08880018.2011.566599.
29. Lim T., Kim S.J., Kim K., et al. Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes. Ann Hematol. 2011;90:1391–8. DOI: 10.1007/s00277-011-1225-0.
30. Akhaddar A., Zalagh M., Belfquih H. Burkitt’s lymphoma: a rare cause of isolated trigeminal neuralgia in a child. Childs Nerv Syst. 2012;28:1125–6. DOI: 10.1007/s00381-012-1735-7.
31. Jiang L., Li Z., Finn L.E., et al. Primary central nervous system B cell lymphoma with fea- tures intermediate between diffuse large B cell lymphoma and Burkitt lymphoma. Int J Clin Exp Pathol. 2012;5(1):72–6.
32. Yoon J.H., Kang H.J., Kim H., et al. Successful Treatment of Primary Central Nervous System Lymphoma without Irradiation in Children: Single Center Experience. J Korean Med Sci. 2012;27:1378–84. DOI: 10.3346/jkms.2012.27.11.1378.
33. Alabdulsalam A., Zaidi S.Z.A., Tailor I., et al. Case Report Primary Burkitt Lymphoma of the Fourth Ventricle in an Immunocompetent Young Patient. Case Rep Pathol. 2014;6:1–6. DOI: 10.1155/2014/630954.
34. Bower K., Shah N. Primary CNS Burkitt Lymphoma: A Case Report of a 55-Year-Old Cerebral Palsy Patient. Case Rep Oncol Med. 2018;2018:1–7. DOI: 10.1155/2018/5869135.
35. Patel P.A., Anand A.S., Parikh S.K., et al. Primary Central Nervous System Burkitt Lymphoma in HIV Positive Pediatric Patient: A Rare Case Report. J Pediatr Neurosci. 2019;14:86–9. DOI: 10.4103/jpn.JPN.
36. Miraclin A., Sivadasan A., Rima S., et al. Pearls & Oy-sters: Primary CNS Burkitt Lymphoma in Pregnancy. Neurology. 2021;96:2141–4. DOI: 10.1212/WNL.0000000000011495.
37. Bahashwan S.M., Barefah A.S., Daous Y.M. Primary Central Nervous System Burkitt Lymphoma , Presenting with Long-Term Fluctuating Level of Consciousness : A Case Report and Literature Review on Challenges in Diagnosis and Management. Am J Case Rep. 2022;23:1–11. DOI: 10.12659/AJCR.936401.
38. Srichawla B.S. Sporadic Burkitt Lymphoma of the Thoracic and Lumbar Spinal Canal in an Adult : Oncogenicity and a Literature Review. Cureus. 2022;14(7):3– 9. DOI: 10.7759/cureus.26860.
39. Soyland D.J., Thanel P.F., Sievers M.E., et al. Primary epidural sporadic Burkitt lymphoma in a 3-year-old: Case report and literature review. Surg Neurol Int. 2022;13(106):1–7. DOI: 10.25259/SNI.
40. Alghamdi A.H., Alzahrani M., Kamal Y.F., et al. Primary CNS Burkitt Lymphoma Presenting as Sudden Bilateral Blindness in a Patient With Underlying Kabuki Syndrome: A Case Report. Cureus. 2024;16(7):7–10. DOI:10.7759/cureus.63725.
41. Rubenstein J.L. Biology of CNS lymphoma and the potential of novel agents. Hematology. 2017;(1):556–64. DOI: 10.1182/asheducation-2017.1.556.
42. Kersten M.J., Kraan W., Doorduijn J., et al. Diffuse large B cell lymphomas relapsing in the CNS lack oncogenic MYD88 and CD79B mutations. Blood Cancer J. 2014;4(12):266–7. DOI: 10.1038/bcj.2014.87.
43. Roland M.M., Anna S., Stefan B., et al. Mutations of CARD11 but not TNFAIP3 may activate the NF- k B pathway in primary CNS lymphoma. Acta Neuropathol. 2010;120:529–35. DOI: 10.1007/s00401-010-0709-7.
44. Ma J., Zhang J., Chen T., et al. Could Cerebral Inflammatory Lesions be the Cellular Origin of Primary Central Nervous System Lymphoma? J Craniofac Surg. 2024;35(4):1209–13. DOI: 10.1097/SCS.0000000000010188.Could.
45. Zhang Y., Zheng S., Li Y., et al. A case of neuropsychiatric lupus with primary central nervous system diffuse large B-cell lymphoma. Front Immunol. 2025;(16):1–6. DOI: 10.3389/fimmu.2025.1636597.
46. Cerchietti L. Genetic mechanisms underlying tumor microenvironment composition and function in diffuse large B-cell lymphoma. Blood. 2024;143(12):1101– 11. DOI: 10.1182/blood.2023021002.
47. Jares P., Colomer D., Campo E. Molecular pathogenesis of mantle cell lymphoma. J Clin Invest. 2012;122(10):3416–23. DOI: 10.1172/JCI61272.3416.
48. Horsman D.E., Gascoyne R.D., Coupland R.W., et al. Comparison of Cytogenetic Analysis, Southern Analysis, and Polymerase Chain Reaction for the Detection of t ( 14 ; 18 ) in Follicular Lymphoma. Hematopathology. 1994;472–8. DOI: 10.1093/ajcp/103.4.472.
49. Janz S., Rabkin C.S. Distribution of t ( 14 ; 18 ) -positive , putative lymphoma precursor cells among B-cell subsets in healthy individuals. Br J Haematol. 2007;138:349–53. DOI: 10.1111/j.1365-2141.2007.06671.x.
50. Janz S., Potter M., Rabkin C.S. Lymphoma- and Leukemia-Associated Chromosomal Translocations in Healthy Individuals. Genes, Chromosom cancer. 2003;223:211–23. DOI: 10.1002/gcc.10178.
51. Roulland S., Navarro J., Grenot P., et al. Follicular lymphoma-like B cells in healthy individuals : a novel intermediate step in early lymphomagenesis. J Exp Med. 2006;203(11):2425–31. DOI: 10.1084/jem.20061292.
52. Schmitz R., Ceribelli M., Pittaluga S., et al. Oncogenic Mechanisms in Burkitt Lymphoma. Cold Spring Harb Perspect Med. 2015;4:1–14.
53. Calvo-Vidal M.N. Zamponi N., Krumsiek J. et al. Oncogenic HSP90 Facilitates Metabolic Alterations in Aggressive B-cell Lymphomas // Cancer Res. 2021. Vol. 81. P. 5202–5216.doi: 10.1158/0008-5472.CAN-21-2734.
54. Smith B.A.H., Deutzmann A., Correa K.M., et al. MYC-driven synthesis of Siglec ligands is a glycoimmune checkpoint // Immunol. Inflamm. 2023. Vol. 120, № 11. P. 1–12.doi: 10.1073/pnas.2215376120.
55. Wang Z., Feng S., Yao X. Molecular pathology of lymphoma and its treatment strategies: from mechanistic elucidation to precision medicine // Front. Immunol. 2025. № 16. P. 1–20.doi: 10.3389/fimmu.2025.1620895.
Review
For citations:
Balayants V.A., Koroleva D.A., Kovrigina A.M., Zvonkov E.E., Kiselev E.O., Obukhova T.N., Yatsyk G.A. Primary central nervous system Burkitt lymphoma: Case report and literature review. Russian journal of hematology and transfusiology. 2025;70(4):530-541. (In Russ.) https://doi.org/10.35754/0234-5730-2025-70-4-530-541
Views:
9
Email this article 
