Central nervous system manifestations of marginal zone B-cell lymphoma

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.

Primary Intracranial Mucosa-Associated Lymphoid Tissue Lymphoma

“…Most patients' clinical signs and symptoms are subtle and insidious, requiring long evaluations and follow-up before the diagnosis becomes clear 9 . Most frequent symptoms include dizziness, fainting spells, nausea, vomiting, memory impairment and blurred vision 9,10 . They may in a few instances show a more rapid progression in a few weeks of evolution.…”

“…They may in a few instances show a more rapid progression in a few weeks of evolution. MALT lymphomas are more frequent between 40 and 50 years of age, with a 5:1 female:male dominance 10 . Dural-based MALT lymphomas are very similar to meningiomas on imaging [10][11][12] .…”

Marginal zone lymphomas involving meningeal dura: possible link to IgG4-related diseases

“…5 Since our original report, several other publications have confirmed a small risk for MALT lymphoma in this site. 7,[32][33][34][35][36][37][38] Among lymphomas primary to the dura, marginal zone lymphoma is reportedly the most common type. However, these cases are still relatively rare and with limited data regarding their clinical behavior.…”

Primary Central Nervous System Mucosa-Associated Lymphoid Tissue Lymphoma-Case Report-

“…18) Only small numbers of CNS MALT lymphomas have been reported. 2,6,7,[11][12][13][15][16][17][18][19]21,22,[24][25][26][27][28]31,33) Most cases of CNS MALT lymphomas occur in middle-aged women in contrast to primary CNS lymphoma which has a slight male predilection, although no specific factors related to sex have been identified. Most patients with CNS MALT lymphoma in the cranium present with insidious onset of headaches, seizure, focal sensory disturbances, and CT/MR imaging findings of well-defined, intracranial, dural masses, which are diagnosed clinically as meningioma.…”

“…MALT lymphoma originating from meningeal tissue is rare, and only a handful of cases and small series have been described. 2,6,7,[11][12][13][15][16][17][18][19]21,22,[24][25][26][27][28]31,33) This subtype is distinct from other primary CNS lymphoma or metastatic CNS lymphoma that can have poor outcomes. Therefore, recognition of this rare subtype of CNS lymphoma is important.…”