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

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.

Primary central nervous system marginal zone lymphoma

“…Given the role played by epithelial cells in the pathogenesis of EMZL, mostly in the stomach, it has been speculated that also meningothelial cells of the arachnoid villi of the venous sinus may play a similar role in PCNSMZL; this hypothesis is further corroborated by the finding that these tumours frequently develop within areas rich in meningothelial cells, such as arachnoidal villi of the dural venous sinuses 20,24–28 …”

“…23 Given the role played by epithelial cells in the pathogenesis of EMZL, mostly in the stomach, it has been speculated that also meningothelial cells of the arachnoid villi of the venous sinus may play a similar role in PCNSMZL; this hypothesis is further corroborated by the finding that these tumours frequently develop within areas rich in meningothelial cells, such as arachnoidal villi of the dural venous sinuses. 20,[24][25][26][27][28] In contrast to PCNSL, there has been no clear association between immunosuppression and the development of PCNSMZL. Only one case of PCNSMZL in an Epstein-Barr virus (EBV)-negative, HCV seropositive liver transplant recipient has been reported.…”

“…17,28,[46][47][48][49] The second most frequent site of involvement is the glomus of the choroid plexus in the lateral ventricles, a structure with ectodermal origin and devoid of a blood-brain barrier. 22,23,25,27,[49][50][51][52] Twenty-eight cases with parenchymal location of this tumour have been reported so far and are mostly localized in the cortex, 6,39,49,[53][54][55][56] basal ganglia, 26,28,[56][57][58] cerebellopontine angle 20,[59][60][61] and retina. 62 The reported onset symptoms in patients with parenchymal PCNSMZL were facial palsy, hemiparesis and memory disturbance.…”

“…74 The second most frequent site of PCNSMZL is the glomus of the choroid plexus in the lateral ventricles and the differential diagnosis for choroid plexus enhancing lesions in addition to lymphoma includes infections, sarcoidosis, papilloma, meningioma, subependymoma and metastases. 22,25,27,50,51 The differential diagnoses considered for the parenchymal localization of PCNSMZL include glioma, intravascular lymphoma and non-neoplastic demyelinating disease. 49 The interesting case of a patient with a long standing history of atypical white matter lesions mimicking multiple sclerosis was reported.…”

Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline

“…3 MZLs rarely involve the central nervous system (CNS), either primarily or secondarily. Most CNS MZL is dural EMZL/MALT lymphoma, with lesions often radiologically indistinguishable meningioma [9][10][11][12][13] ; however, MRI may distinguish from meningioma by a more prominent dural tail. 14 Histological confirmation has important implications for prognosis and management, as dural EMZL/MALT lymphoma has a better prognosis than high-grade primary central nervous system lymphomas.…”