Abstract
Primary or secondary central nervous system (CNS) involvement by marginal zone B-cell lymphoma (MZBCL) is rare. A retrospective analysis of patients was done with MZBCL involving the CNS, diagnosed and treated at our institution between 2004 and 2010. We identified 10 MZBCL patients with primary (six) or secondary (four) CNS involvement. Five patients presented with primary dural lymphoma and were treated with surgical resection, whole-brain radiation, or systemic chemotherapy. Only one patient had CNS relapse 5 years later. A single patient with primary intraocular lymphoma achieved clinical remission with ocular radiotherapy and systemic chemotherapy. Four patients had ocular MZBCL within 5 years of the initial diagnosis of primary ocular adnexal MZBCL and primary splenic MZBCL. There was no evidence of local recurrence in all but one who developed systemic relapse after 3 years of follow-up. Primary or secondary CNS involvement by MZBCL display indolent clinical behavior and have a generally favorable prognosis, underlining the importance of their differentiation from aggressive lymphomas that more commonly involve the CNS.
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Acknowledgements
The authors thank Dr. Gerald Byrne from the Department of Hematopathology for his pathology review of the cases.
Funding
This work was supported by the United States Public Health Service, National Institutes of Health [grant numbers R01-CA109335, R01-CA122105], and the Dwoskin Family Foundation.
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Bayraktar, S., Stefanovic, A., Montague, N. et al. Central nervous system manifestations of marginal zone B-cell lymphoma. Ann Hematol 89, 1003–1009 (2010). https://doi.org/10.1007/s00277-010-0976-3
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DOI: https://doi.org/10.1007/s00277-010-0976-3