Clinicopathological Features and Predictors of Survival in Primary CNS Lymphoma
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Abstract
Background: Primary CNS lymphoma (PCNSL) is a rare form of extranodal lymphoma, representing 3 to 4% of brain tumors. We aim to report the clinicopathological features and predictors of survival in patients with PCNSL.
Materials and Methods: This retrospective study was conducted at Shifa International Hospital, Pakistan. Patients diagnosed with PCNSL from 2018 to 2023 were included. The Hans algorithm was used to further categorize cases of high-grade lymphoma. Data were analyzed using IBM® SPSS Statistics Software Version 26.
Results: Fifty-two patients were diagnosed with PCNSL during the study period, out of whom twenty-seven patients had complete clinical and laboratory data available. From these 27 patients, 14 (52%) were male. Mean age was 48.78 ± 13.46 years. Twenty-four (88.9%) patients were immunocompetent and none had HIV infection. On radiology, a solitary lesion was seen in 19 (70.4%) patients with parietal lobe being the most common location in 8 patients. Twenty-six patients were diagnosed with high-grade non-Hodgkin B-cell lymphoma whereas one patient had low-grade non-Hodgkin B-cell lymphoma. Seven patients expired, with median survival of five months (range 1–31 months). Ki-67 proliferative index >80% was the only factor identified as significantly associated with decreased overall survival (p<0.05).
Conclusion: PCNSL is a rare and aggressive disease. High Ki-67 proliferative index is a significant predictor of overall survival in these patients. Lack of association of HIV, frequent occurrence of Bcl6 positivity and a single case of low-grade lymphoma were unique features identified in our study population.
2. Villano JL, Koshy M, Shaikh H, et al. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer. 2011;105(9):1414–1418.
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(7):1720-48.
4. Grommes C, DeAngelis LM. Primary CNS Lymphoma. J Clin Oncol. 2017;35(21):2410-2418.
5. Lukas RV, Riedell P, Horowitz PM, et al. Diagnostic Evaluation in Primary CNS Lymphoma. Neurologist. 2018;23(2):53-54.
6. Scott BJ, Douglas VC, Tihan T, et al. A systematic approach to the diagnosis of suspected central nervous system lymphoma. JAMA neurology. 2013 1;70(3):311-9.
7. Hattab EM, Martin SE, Al-Khatib SM, et al. Most primary central nervous system diffuse large B-cell lymphomas occurring in immunocompetent individuals belong to the nongerminal center subtype: a retrospective analysis of 31 cases. Modern Pathology. 2010;23(2):235-43.
8. Citterio G, Reni M, Gatta G, et al. Primary central nervous system lymphoma. Crit Rev Oncol Hematol. 2017;113:97-110.
9. Momota H, Narita Y, Maeshima AM, et al. Prognostic value of immunohistochemical profile and response to high-dose methotrexate therapy in primary CNS lymphoma. Journal of neuro-oncology. 2010;98:341-8.
10. Chang CC, Kampalath B, Schultz C, et al. Expression of p53, c-Myc, or Bcl-6 suggests a poor prognosis in primary central nervous system diffuse large B-cell lymphoma among immunocompetent individuals. Archives of pathology & laboratory medicine. 2003;127(2):208-12.
11. Hans CP, Weisenburger DD, Greiner TC, et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Blood. 2004;103(1):275-82.
12. He X, Chen Z, Fu T, et al. Ki-67 is a valuable prognostic predictor of lymphoma but its utility varies in lymphoma subtypes: evidence from a systematic meta-analysis. BMC cancer. 2014;14:1-3.
13. Cho U, Oh WJ, Hong YK, et al. Prognostic significance of high ki-67 index and histogenetic subclassification in primary central nervous system lymphoma. Applied immunohistochemistry & molecular morphology. 2018;26(4):254-62.
14. Phalak P, Rajamma BM, Pradeep A, et al. Prognostic significance of immunohistochemical markers in Primary CNS lymphomas. IP Arch Cytol Histopathol Res. 2023;6(3):160-5.
15. Preusser M, Woehrer A, Koperek O, et al. Primary central nervous system lymphoma: a clinicopathological study of 75 cases. Pathology. 2010;42(6):547-52.
16. Levy O, DeAngelis LM, Filippa DA, et al. Bcl‐6 predicts improved prognosis in primary central nervous system lymphoma. Cancer: Interdisciplinary International Journal of the American Cancer Society. 2008;112(1):151-6.
17. Kaji FA, Martinez‐Calle N, Sovani V, et al. Rare central nervous system lymphomas. Br J Haematol. 2022;197(6):662-78.
18. Abbi KK, Muzaffar M, Gaudin D, et al. Primary CNS lymphoplasmacytic lymphoma: a case report and review of literature. Hematology/Oncology and Stem Cell Therapy. 2013;6(2):76-8.
19. Lim T, Kim SJ, Kim K, et al. Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes. Annals of Hematology. 2011;90:1391-8.
20. Woo HY, Chae SW, DO SI, et al. Clinicopathological Characterization of Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System. Anticancer Research. 2022;42(11):5601-8.
21. Meyer PN, Fu K, Greiner TC, et al. Immunohistochemical methods for predicting cell of origin and survival in patients with diffuse large B-cell lymphoma treated with rituximab. Journal of clinical oncology. 2011;29(2):200-7.
22. Brandsma D, Bromberg JE. Primary CNS lymphoma in HIV infection. Handbook of clinical neurology. 2018;152:177-86.
23. Sierra del Rio M, Rousseau A, Soussain C, et al. Primary CNS lymphoma in immunocompetent patients. The oncologist. 2009;14(5):526-39.
24.
25. Ahmed A, Hashmi FK, Khan GM. HIV outbreaks in Pakistan. The Lancet HIV. 2019;6(7):e418.
| Files | ||
| Issue | Vol 20 No 3 (2026) | |
| Section | Original Article(s) | |
| Keywords | ||
| Acute lymphoblastic leukemia; Bisulfite sanger sequencing; Epigenetics; MAPK1; Promoter-methylation | ||
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