Follicular Lymphoma with Cranial Nerve and Central Nervous System Involvement Successfully Treated with Bendamustine and Rituximab: A Case Report and Literature Review
Main Article Content
Abstract
Follicular lymphoma is the most common indolent non- Hodgkin lymphoma (NHL) and accounts for approximately 20-30% of all NHL cases in Western countries. In general, systemic therapies are not considered to be curative. Despite this, prognosis is excellent with a 5-year relative survival rate of 90% and median overall survival rates ranging from 10-20 years across numerous studies. The exact incidence of central nervous system (CNS) involvement in cases of follicular lymphoma is unknown, however retrospective data suggest an incidence of 0.2% though this likely represents an underestimation of the true incidence. The optimum treatment approach for CNS disease in follicular lymphoma is poorly defined and no randomized studies exist which prove the superiority of one approach over another. Retrospective data suggest that resection, with or without adjuvant radiation, is an effective strategy for patients with local disease limited to the CNS. In cases where synchronous systemic disease is present, treatment regimens incorporating high-dose methotrexate and bendamustine or anthracycline-based chemotherapy have demonstrated efficacy. We report a case of a 59-year-old female with stage IV follicular lymphoma with synchronous systemic and leptomeningeal involvement who achieved complete remission following treatment with bendamustine, rituximab, and intra-thecal
methotrexate. More long-term follow up data are needed to better characterize the prognosis for patients with CNS involvement who obtain complete remission following initial therapies. Treatment selection for patients with CNS involvement should take into account the presence or absence of systemic disease, patient comorbidities and performance status, and the likelihood of transformation to a high-grade process.
Keywords:
Bendamustine, Chemoimmunotherapy, Follicular Lymphoma, Lymphoproliferative Disorders, Neurolymphomatosis, Non-Hodgkin Lymphoma, Rituximab, Secondary CNS Lymphoma
Article Details
How to Cite
ROBERTS, Nathan et al.
Follicular Lymphoma with Cranial Nerve and Central Nervous System Involvement Successfully Treated with Bendamustine and Rituximab: A Case Report and Literature Review.
Medical Research Archives, [S.l.], v. 12, n. 5, may 2024.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/5379>. Date accessed: 30 june 2024.
doi: https://doi.org/10.18103/mra.v12i5.5379.
Section
Review Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. Carbone A, Roulland S, Gloghini A, et al. Follicular lymphoma. Nat Rev Dis Primers. 2019;5(1):83.
2. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2015. CA Cancer J Clin. 2015;65(1):5-29.
3. Solal-Céligny P, Roy P, Colombat P, et al. Follicular lymphoma international prognostic index. Blood. 2004;104(5):1258-1265.
4. Federico M, Bellei M, Marcheselli L, et al. Follicular lymphoma international prognostic index 2: a new prognostic index for follicular lymphoma developed by the international follicular lymphoma prognostic factor project. J Clin Oncol. 2009;27(27):4555-4562.
5. Pastore A, Jurinovic V, Kridel R, et al. Integration of gene mutations in risk prognostication for patients receiving first-line immunochemotherapy for follicular lymphoma: a retrospective analysis of a prospective clinical trial and validation in a population-based registry. Lancet Oncol. 2015;16(9):1111-1122.
6. Bachy E, Maurer MJ, Habermann TM, et al. A simplified scoring system in de novo follicular lymphoma treated initially with immunochemotherapy. Blood. 2018;132(1):49-58.
7. Rodríguez-Sevilla JJ, Fernández-Rodríguez C, Bento L, et al. Evaluation of 4 prognostic indices in follicular lymphoma treated in first line with immunochemotherapy. Blood Adv. 2023;7(8):1606-1614.
8. Friedberg J, Huang J, Dillon H, et al. Initial therapeutic strategy infollicular lymphoma: an analysis from the National LymphoCareStudy.J Clin Oncol. 2006;24:428s.
9. Advani R, Rosenberg SA, Horning SJ. Stage I and II follicular non-Hodgkin's lymphoma: long-term follow-up of no initial therapy. J Clin Oncol. 2004;22(8):1454-1459.
10. Ardeshna KM, Smith P, Norton A, et al. Long-term effect of a watch and wait policy versus immediate systemic treatment for asymptomatic advanced-stage non-Hodgkin lymphoma: a randomised controlled trial. Lancet. 2003;362(9383):516-522.
11. Brice P, Bastion Y, Lepage E, et al. Comparison in low-tumor-burden follicular lymphomas between an initial no-treatment policy, prednimustine, or interferon alfa: a randomized study from the Groupe d'Etude des Lymphomes Folliculaires. Groupe d'Etude des Lymphomes de l'Adulte. J Clin Oncol. 1997;15(3):1110-1117.
12. Schmitz N, Zeynalova S, Nickelsen M, et al. CNS international prognostic index: a risk model for CNS relapse in patients With diffuse large B-cell lymphoma treated with R-CHOP. J Clin Oncol. 2016;34(26):3150-3156.
13. Ferreri AJM, Doorduijn JK, Re A, et al. MATRix-RICE therapy and autologous haematopoietic stem-cell transplantation in diffuse large B-cell lymphoma with secondary CNS involvement (MARIETTA): an international, single-arm, phase 2 trial. Lancet Haematol. 2021;8(2):e110-e121.
14. Korfel A, Elter T, Thiel E, et al. Phase II study of central nervous system (CNS)-directed chemotherapy including high-dose chemotherapy with autologous stem cell transplantation for CNS relapse of aggressive lymphomas. Haematologica. 2013;98(3):364-370.
15. Ferreri AJ, Donadoni G, Cabras MG, et al. High doses of antimetabolites followed by high-dose sequential chemoimmunotherapy and autologous stem-cell transplantation in patients with systemic B-cell lymphoma and secondary CNS involvement: final results of a multicenter phase II trial. J Clin Oncol. 2015;33(33):3903-3910.
16. Pophali PA, Thanarajasingam G, Pulido J, Johnston PB, Go RS. Low grade b-cell non-Hodgkin lymphomas (NHL) involving the central nervous system (CNS): an analysis from the National Cancer Database (NCDB) Journal of Clinical Oncology. 2017;35(15):p. e13083.
17. Ando T, Kamoshita S, Riku Y, et al. Neurolymphomatosis in follicular lymphoma: an autopsy case report. Neuropathology. 2022;42(4):295-301.
18. Costa R, Costa R, Costa R. Follicular lymphoma presenting with leptomeningeal disease. Case Rep Hematol. 2014;2014:767621.
19. Khurana A, Novo M, Nowakowski GS, et al. Clinical manifestations of, diagnostic approach to, and treatment of neurolymphomatosis in the rituximab era [published correction appears in Blood Adv. 2021 Apr 13;5(7):2053]. Blood Adv. 2021;5(5):1379-1387.
20. DeVries AH, Howe BM, Spinner RJ, Broski SM. B-cell peripheral neurolymphomatosis: MRI and 18F-FDG PET/CT imaging characteristics. Skeletal Radiol. 2019;48(7):1043-1050.
21. Kinoshita H, Yamakado H, Kitano T, et al. Diagnostic utility of FDG-PET in neurolymphomatosis: report of five cases. J Neurol. 2016;263(9):1719-1726.
22. Beriwal S, Hou JS, Miyamoto C, Garcia-Young JA. Primary dural low grade BCL-2 negative follicular lymphoma: a case report. J Neurooncol. 2003;61(1):23-25.
23. Hamilton DK, Bourne TD, Ahmed H, Cousar JB, Mandell JW, Sheehan JP. Follicular lymphoma of the dura: case report. Neurosurgery. 2006;59(3):E703-E704.
24. Low I, Allen J. Low-grade follicular lymphoma in the dura: rare mimic of meningioma. Neuropathology. 2006;26(6):564-568.
25. Riccioni L, Morigi F, Cremonini AM. Follicular lymphoma of the dura associated with meningioma: a case report and review of the literature. Neuropathology. 2007;27(3):278-283.
26. Peltier J, Fichten A, Lefranc M, et al. Lymphome folliculaire méningé. A propos d'un cas [Follicular dural lymphoma. Case report]. Neurochirurgie. 2009;55(3):345-349.
27. Tandon R, Mukherjee U, Abrari A, Patir R, Tandon A, Bansal B. Primary dural follicular lymphoma masquerading as meningioma: a case report. Br J Neurosurg. 2012;26(6):905-906.
28. Yamaguchi J, Kato S, Iwata E, et al. Pediatric-type follicular lymphoma in the dura: a case report and literature review. World Neurosurg. 2018;115:176-180.
29. MacCann R, Gleeson JP, Aird JJ, et al. An unusual presentation of a low grade follicular lymphoma masquerading as a meningioma. Case Report Ann Hematol Oncol. 2018;5(6):121
30. Altshuler E, Richhart R, Iqbal U, Chaffin J. Dural follicular lymphoma: case report and literature review. J Investig Med High Impact Case Rep. 2021;9:23247096211056768.
31. Spectre G, Gural A, Amir G, Lossos A, Siegal T, Paltiel O. Central nervous system involvement in indolent lymphomas. Ann Oncol. 2005;16(3):450-454.
32. Grupka NL, Seinfeld J, Ryder J, Lillehei KO, Kleinschmidt-Demasters BK. Secondary central nervous system involvement by follicular lymphoma: case report and review of the literature. Surg Neurol. 2006;65(6):590-594.
33. Karadurmus N, Ataergin S, Erdem G, et al. A rare presentation of follicular lymphoma: cerebellar involvement, successfully treated with a combination of radiotherapy and chemotherapy. Cancer Res Treat. 2013;45(3):234-238.
34. Tsuboi Y, Shimizu M, Kuroda A, Suyama T, Seki M, Shinagawa A. Follicular lymphoma with secondary central nervous system relapse: a case report and literature review. Oxf Med Case Reports. 2023;2023(11):omad118. Published 2023 Nov 28.
35. Kim T, Choi HY, Lee HS, et al. Clinical response and pharmacokinetics of bendamustine as a component of salvage R-B(O)AD therapy for the treatment of primary central nervous system lymphoma (PCNSL). BMC Cancer. 2018;18(1):729.
36. Morschhauser F, Tilly H, Chaidos A, et al. Tazemetostat for patients with relapsed or refractory follicular lymphoma: an open-label, single-arm, multicentre, phase 2 trial. Lancet Oncol. 2020;21(11):1433-1442.
37. Budde LE, Sehn LH, Matasar M, et al. Safety and efficacy of mosunetuzumab, a bispecific antibody, in patients with relapsed or refractory follicular lymphoma: a single-arm, multicentre, phase 2 study. Lancet Oncol. 2022;23(8):1055-1065.
38. Fowler NH, Dickinson M, Dreyling M, et al. Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial. Nat Med. 2022;28(2):325-332.
39. Jacobson CA, Chavez JC, Sehgal AR, et al. Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial. Lancet Oncol. 2022;23(1):91-103.
40. Cook MR, Dorris CS, Makambi KH, et al. Toxicity and efficacy of CAR T-cell therapy in primary and secondary CNS lymphoma: a meta-analysis of 128 patients. Blood Adv. 2023;7(1):32-39.
2. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2015. CA Cancer J Clin. 2015;65(1):5-29.
3. Solal-Céligny P, Roy P, Colombat P, et al. Follicular lymphoma international prognostic index. Blood. 2004;104(5):1258-1265.
4. Federico M, Bellei M, Marcheselli L, et al. Follicular lymphoma international prognostic index 2: a new prognostic index for follicular lymphoma developed by the international follicular lymphoma prognostic factor project. J Clin Oncol. 2009;27(27):4555-4562.
5. Pastore A, Jurinovic V, Kridel R, et al. Integration of gene mutations in risk prognostication for patients receiving first-line immunochemotherapy for follicular lymphoma: a retrospective analysis of a prospective clinical trial and validation in a population-based registry. Lancet Oncol. 2015;16(9):1111-1122.
6. Bachy E, Maurer MJ, Habermann TM, et al. A simplified scoring system in de novo follicular lymphoma treated initially with immunochemotherapy. Blood. 2018;132(1):49-58.
7. Rodríguez-Sevilla JJ, Fernández-Rodríguez C, Bento L, et al. Evaluation of 4 prognostic indices in follicular lymphoma treated in first line with immunochemotherapy. Blood Adv. 2023;7(8):1606-1614.
8. Friedberg J, Huang J, Dillon H, et al. Initial therapeutic strategy infollicular lymphoma: an analysis from the National LymphoCareStudy.J Clin Oncol. 2006;24:428s.
9. Advani R, Rosenberg SA, Horning SJ. Stage I and II follicular non-Hodgkin's lymphoma: long-term follow-up of no initial therapy. J Clin Oncol. 2004;22(8):1454-1459.
10. Ardeshna KM, Smith P, Norton A, et al. Long-term effect of a watch and wait policy versus immediate systemic treatment for asymptomatic advanced-stage non-Hodgkin lymphoma: a randomised controlled trial. Lancet. 2003;362(9383):516-522.
11. Brice P, Bastion Y, Lepage E, et al. Comparison in low-tumor-burden follicular lymphomas between an initial no-treatment policy, prednimustine, or interferon alfa: a randomized study from the Groupe d'Etude des Lymphomes Folliculaires. Groupe d'Etude des Lymphomes de l'Adulte. J Clin Oncol. 1997;15(3):1110-1117.
12. Schmitz N, Zeynalova S, Nickelsen M, et al. CNS international prognostic index: a risk model for CNS relapse in patients With diffuse large B-cell lymphoma treated with R-CHOP. J Clin Oncol. 2016;34(26):3150-3156.
13. Ferreri AJM, Doorduijn JK, Re A, et al. MATRix-RICE therapy and autologous haematopoietic stem-cell transplantation in diffuse large B-cell lymphoma with secondary CNS involvement (MARIETTA): an international, single-arm, phase 2 trial. Lancet Haematol. 2021;8(2):e110-e121.
14. Korfel A, Elter T, Thiel E, et al. Phase II study of central nervous system (CNS)-directed chemotherapy including high-dose chemotherapy with autologous stem cell transplantation for CNS relapse of aggressive lymphomas. Haematologica. 2013;98(3):364-370.
15. Ferreri AJ, Donadoni G, Cabras MG, et al. High doses of antimetabolites followed by high-dose sequential chemoimmunotherapy and autologous stem-cell transplantation in patients with systemic B-cell lymphoma and secondary CNS involvement: final results of a multicenter phase II trial. J Clin Oncol. 2015;33(33):3903-3910.
16. Pophali PA, Thanarajasingam G, Pulido J, Johnston PB, Go RS. Low grade b-cell non-Hodgkin lymphomas (NHL) involving the central nervous system (CNS): an analysis from the National Cancer Database (NCDB) Journal of Clinical Oncology. 2017;35(15):p. e13083.
17. Ando T, Kamoshita S, Riku Y, et al. Neurolymphomatosis in follicular lymphoma: an autopsy case report. Neuropathology. 2022;42(4):295-301.
18. Costa R, Costa R, Costa R. Follicular lymphoma presenting with leptomeningeal disease. Case Rep Hematol. 2014;2014:767621.
19. Khurana A, Novo M, Nowakowski GS, et al. Clinical manifestations of, diagnostic approach to, and treatment of neurolymphomatosis in the rituximab era [published correction appears in Blood Adv. 2021 Apr 13;5(7):2053]. Blood Adv. 2021;5(5):1379-1387.
20. DeVries AH, Howe BM, Spinner RJ, Broski SM. B-cell peripheral neurolymphomatosis: MRI and 18F-FDG PET/CT imaging characteristics. Skeletal Radiol. 2019;48(7):1043-1050.
21. Kinoshita H, Yamakado H, Kitano T, et al. Diagnostic utility of FDG-PET in neurolymphomatosis: report of five cases. J Neurol. 2016;263(9):1719-1726.
22. Beriwal S, Hou JS, Miyamoto C, Garcia-Young JA. Primary dural low grade BCL-2 negative follicular lymphoma: a case report. J Neurooncol. 2003;61(1):23-25.
23. Hamilton DK, Bourne TD, Ahmed H, Cousar JB, Mandell JW, Sheehan JP. Follicular lymphoma of the dura: case report. Neurosurgery. 2006;59(3):E703-E704.
24. Low I, Allen J. Low-grade follicular lymphoma in the dura: rare mimic of meningioma. Neuropathology. 2006;26(6):564-568.
25. Riccioni L, Morigi F, Cremonini AM. Follicular lymphoma of the dura associated with meningioma: a case report and review of the literature. Neuropathology. 2007;27(3):278-283.
26. Peltier J, Fichten A, Lefranc M, et al. Lymphome folliculaire méningé. A propos d'un cas [Follicular dural lymphoma. Case report]. Neurochirurgie. 2009;55(3):345-349.
27. Tandon R, Mukherjee U, Abrari A, Patir R, Tandon A, Bansal B. Primary dural follicular lymphoma masquerading as meningioma: a case report. Br J Neurosurg. 2012;26(6):905-906.
28. Yamaguchi J, Kato S, Iwata E, et al. Pediatric-type follicular lymphoma in the dura: a case report and literature review. World Neurosurg. 2018;115:176-180.
29. MacCann R, Gleeson JP, Aird JJ, et al. An unusual presentation of a low grade follicular lymphoma masquerading as a meningioma. Case Report Ann Hematol Oncol. 2018;5(6):121
30. Altshuler E, Richhart R, Iqbal U, Chaffin J. Dural follicular lymphoma: case report and literature review. J Investig Med High Impact Case Rep. 2021;9:23247096211056768.
31. Spectre G, Gural A, Amir G, Lossos A, Siegal T, Paltiel O. Central nervous system involvement in indolent lymphomas. Ann Oncol. 2005;16(3):450-454.
32. Grupka NL, Seinfeld J, Ryder J, Lillehei KO, Kleinschmidt-Demasters BK. Secondary central nervous system involvement by follicular lymphoma: case report and review of the literature. Surg Neurol. 2006;65(6):590-594.
33. Karadurmus N, Ataergin S, Erdem G, et al. A rare presentation of follicular lymphoma: cerebellar involvement, successfully treated with a combination of radiotherapy and chemotherapy. Cancer Res Treat. 2013;45(3):234-238.
34. Tsuboi Y, Shimizu M, Kuroda A, Suyama T, Seki M, Shinagawa A. Follicular lymphoma with secondary central nervous system relapse: a case report and literature review. Oxf Med Case Reports. 2023;2023(11):omad118. Published 2023 Nov 28.
35. Kim T, Choi HY, Lee HS, et al. Clinical response and pharmacokinetics of bendamustine as a component of salvage R-B(O)AD therapy for the treatment of primary central nervous system lymphoma (PCNSL). BMC Cancer. 2018;18(1):729.
36. Morschhauser F, Tilly H, Chaidos A, et al. Tazemetostat for patients with relapsed or refractory follicular lymphoma: an open-label, single-arm, multicentre, phase 2 trial. Lancet Oncol. 2020;21(11):1433-1442.
37. Budde LE, Sehn LH, Matasar M, et al. Safety and efficacy of mosunetuzumab, a bispecific antibody, in patients with relapsed or refractory follicular lymphoma: a single-arm, multicentre, phase 2 study. Lancet Oncol. 2022;23(8):1055-1065.
38. Fowler NH, Dickinson M, Dreyling M, et al. Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial. Nat Med. 2022;28(2):325-332.
39. Jacobson CA, Chavez JC, Sehgal AR, et al. Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial. Lancet Oncol. 2022;23(1):91-103.
40. Cook MR, Dorris CS, Makambi KH, et al. Toxicity and efficacy of CAR T-cell therapy in primary and secondary CNS lymphoma: a meta-analysis of 128 patients. Blood Adv. 2023;7(1):32-39.