Primary intracranial marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle: Case report and review of pathogenesis.

Content Provider	Europe PMC
Author	Hajtovic, Sabastian Yu, Edward Bershadskiy, Alexander Sacho, Raphael Gilad, Ronit
Copyright Year	2022
Abstract	Background: Primary central nervous system lymphoma (PCNSL) is an aggressive extranodal subtype of nonHodgkin’s lymphoma. Ventricle-predominant PCNSL, arising in the CNS ventricular system, is a rare entity. In over 90% of cases, PCNSL is classified as diffuse large B-cell lymphoma. Rarely, PCNSL may be classified as marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (MALT). Taken together, a primary MALT-type MZBCL arising in a cerebral ventricle is an extremely rare presentation.Case Description: A 69-year-old female presented with a persistent left frontal headache for 1 year. Magnetic resonance imaging revealed an enhancing soft-tissue lesion within the left lateral ventricle, with associated periventricular edema. We performed an excisional biopsy of the tumor, which grossly had the appearance of a meningioma. Histopathology of the tumor was consistent with MZBCL of the MALT type. The patient was treated with Rituximab and Ibrutinib. Six months after surgery, she remained neurologically intact and free of disease.Conclusion: We report the case of a primary MALT-type MZBCL arising in the CNS ventricular system, with characteristics mimicking meningioma. This lymphoma involved the lateral ventricle and likely originated from the choroid plexus. Meningothelial cells and epithelial cells in the choroid plexus may acquire MALT in response to chronic inflammatory stimuli, such as infection or autoimmune disease. In rare cases, MALT lymphoma may develop as part of this pathogenesis.
ISSN	22295097
Volume Number	13
PubMed Central reference number	PMC9062969
PubMed reference number	35509545
Journal	Surgical Neurology International [Surg Neurol Int]
e-ISSN	21527806
DOI	10.25259/SNI_54_2022
Language	English
Publisher	Scientific Scholar
Publisher Date	2022-04-29
Publisher Place	USA
Access Restriction	Open
Rights License	This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. Copyright: © 2022 Surgical Neurology International
Subject Keyword	Cerebral ventricle Choroid plexus CNS lymphoma MALT lymphoma Marginal zone B-cell lymphoma
Content Type	Text
Resource Type	Article
Subject	Neurology (clinical) Surgery