IBCL-417 Primary Intracranial Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue in Cerebral Localization: Case Report

• Published in: Clinical lymphoma, myeloma and leukemia Vol. 23; p. S453
• Main Authors: Tlohi, Kawtar, Benmoussa, Amine, Lamchahab, Mouna, Rachid, Mohamed, Qachouh, Meryem, Khoubila, Nissrine, Cherkaoui, Siham, Madani, Abdellah
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-09-2023
• Subjects: cerebral localization; CNS lymphoma; IBCL; MALT lymphoma; marginal zone B-cell lymphoma; multidisciplinary treatment; IBCL; cerebral localization; MALT lymphoma; marginal zone B-cell lymphoma; CNS lymphoma; multidisciplinary treatment; case
• ISSN: 2152-2650; 2152-2669
• DOI: 10.1016/S2152-2650(23)01360-5

Primary central nervous system lymphoma (PCNSL) is an aggressive extra nodal subtype of non-Hodgkin lymphoma, with over 90% of cases classified as diffuse large B-cell lymphoma. However, the occurrence of PCNSL as a marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (MALT) is rare, especially in cerebral localization.
A 61-year-old female presented with a persistent frontal headache and generalized seizures over a 4-month period. Magnetic resonance imaging revealed an enhancing bifrontal extra-axial mass. An excisional biopsy confirmed the diagnosis of MZBCL of the MALT type. The patient received treatment with rituximab and bendamustine for 6 cycles. At 8 months of follow-up, she remained neurologically intact and showed no evidence of disease.
Primary cerebral MZL is a rare and indolent low-grade CNS lymphoma with a relatively favorable prognosis. Treatment typically involves a multidisciplinary approach, including surgical intervention to relieve brain compression, along with low to moderate doses of radiotherapy and/or systemic chemotherapy.