Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Ariani, Rojine; Ballas, Leslie K.

doi:10.1159/000515780

Cited by 7 publications

(7 citation statements)

References 15 publications

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“…Also involvement of the CNS by marginal zone lymphoma is extremely rare. Primary cerebral marginal zone lymphoma has most commonly been described in association with the dura mater 17 19–22. The neurological symptoms of our patient (paresis left leg, impaired coordination left hand and seizures) were related to his white matter lesion in the right parietal lobe.…”

Section: Discussionmentioning

confidence: 57%

“…One case report describes a patient with AL amyloidoma where the lesion was not feasible to resection 7. A recently published case report describes two patients with primary CNS marginal zone lymphoma successfully treated with radiotherapy 17. Another case of a patient with dural marginal zone lymphoma and amyloid depositions responded well to radiotherapy too 18.…”

Section: Discussionmentioning

confidence: 99%

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Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis

Traets,

Chuwonpad,

Leguit

et al. 2024

BMJ Case Rep

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A man in his 60s, known with multiple sclerosis, presented with seizures and paresis of the left arm and leg. Brain imaging showed a white matter lesion, right parietal, which was progressive over the last 6 years and not typical for multiple sclerosis. Brain biopsy showed a B-cell infiltrate with IgA lambda monotypic plasma cell differentiation and amyloid deposits, typed as lambda immunoglobulin light chain (AL). Bone marrow biopsy and PET/CT ruled out a systemic lymphoma. Extended history taking, blood and urine testing (including cardiac biomarkers) identified no evidence of systemic amyloidosis-induced organ dysfunction.Primary cerebral AL amyloidoma is a very rare entity where optimal treatment is difficult to assess. The patient was treated with locally applied volumetric modulated arc radiotherapy, 24 Gy, divided in 12 fractions. Afterwards, the paresis of the left arm partially resolved, and the function of the left leg improved. Seizures did not occur anymore.

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis

Traets,

Chuwonpad,

Leguit

et al. 2024

BMJ Case Rep

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“…The possible mechanisms behind the pathogenesis of CNS MZBL could be represented by a localization of an undetected primary extracranial MZBL or a chronic in ammation. This last condition could derive from a direct chronic antigenic stimulation of the dura, for example for a long-standing history of untreated HBV [12,13] or HCV [14]. One patient with CNS MZBL had a long history of white matter disease with some features of multiple sclerosis [5] and another patient was suffering from a Chlamydia psittaci infection [15].…”

Section: Case and Literature Discussionmentioning

confidence: 99%

Primary CNS extranodal marginal zone B-cell lymphoma: an unusual localization and clinical presentation

Fiorentino

Pizzimenti

Pierconti

et al. 2023

Preprint

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Background Primary Central Nervous System (CNS) extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma, characterized predominantly by small B cells, plasma cells, monocytoid cells, and scattered large immunoblasts. Primary CNS MZBL is a slow-growing tumour that remains localized and is characterized by an excellent clinical prognosis. Case presentation: A 48-year-old HIV-negative female with a history of a head trauma a year earlier, presented with worsening neurological symptoms and an MRI finding of an extra-axial mass of about 3 cm within the left lateral ventricle. From histopathology and immunohistochemistry, the lesion was diagnosed as a CNS MZBL: since no other primary lesions were found, the base of the chorioid plexuses of the left lateral ventricle was considered the primary site. Conclusions The current case is the first literature reported primary CNS MZBL arising in this anatomical site and paves the way for further studies on the role of chronic inflammation (in our case resulting from a trauma) in the pathogenesis not only of primary CNS MZBLs, but also of lymphomas in general. Additionally, it could be a starting point for studies analyzing the role of meningothelial cells in the pathogenesis of primary CNS MZBL.

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“…The possible mechanisms underlying the pathogenesis of CNS MZBL could be ascribed to an implantation metastasis of undetected or vanishing meningeal MZBL (13,14), a localisation of an undetected primary extracranial MZBL, or chronic inflammation. This last condition could derive from a direct chronic antigenic stimulation of the dura, for example, from a long-standing history of untreated HBV (15,16) or HCV (17). One patient with CNS MZBL had a long history of white matter disease with some features of multiple sclerosis (5), and another suffered from a Chlamydia psittaci infection (18).…”

Section: Discussionmentioning

confidence: 99%

Unusual localization and clinical presentation of primary central nervous system extranodal marginal zone B‑cell lymphoma: A case report

et al. 2023

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Primary central nervous system (CNS) extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma, characterised predominantly by small B cells, plasma cells, monocytoid cells and scattered large immunoblasts. Primary CNS MZBL is a slow-growing tumour that remains localised and is characterised by an excellent clinical prognosis. The present study describes the case of a 48-year-old HIV-negative female patient with a history of head trauma 1 year prior, who presented with worsening neurological symptoms and a magnetic resonance imaging finding of a ~3-cm extra-axial mass within the left lateral ventricle. From histopathology and immunohistochemistry, the lesion was diagnosed as a CNS MZBL; as no other primary lesions were found, the base of the choroid plexuses of the left lateral ventricle was considered the primary site. To the best of our knowledge, the current case is the first study to report on primary CNS MZBL arising in this anatomical site and paves the way for further studies on the role of chronic inflammation (in the present case resulting from trauma) in the pathogenesis not only of primary CNS MZBL but also of lymphoma in general. Additionally, this report could serve as a starting point for studies analysing the role of meningothelial cells in the pathogenesis of primary CNS MZBL.

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Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Cited by 7 publications

References 15 publications

Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis

Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis

Primary CNS extranodal marginal zone B-cell lymphoma: an unusual localization and clinical presentation

Unusual localization and clinical presentation of primary central nervous system extranodal marginal zone B‑cell lymphoma: A case report

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