Primary central nervous system marginal zone B‐cell lymphoma arising from the dural meninges: A case report and review of literature

Abstract

Primary central nervous system (CNS) marginal zone B‐cell lymphoma (MZBCL) arising from the dural meninges is a rare but indolent disease. This malignancy can present in various ways, hence making it difficult to diagnose. Biopsy results dictate an appropriate treatment plan, which commonly consists of a combination of surgical resection, whole brain radiotherapy and systemic therapy.

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

“…Biopsy of the lesion is crucial in the definitive diagnosis of MZBL. As in our presented cases, tumors have been found to be composed of B cells positive for CD20 and clonal rearrangements of IgH and k, without expression of CD5, CD10, CD43, Bcl-6, and cyclin D1 [7,10]. The presence of a low proliferation index in these cells is characteristic of the indolent nature of CNS MZBL.…”

“…Although there is currently no consensus on the optimal course of treatment of primary CNS MZBL due to the limited number of cases described in the available literature, the use of RT has been demonstrated to increase overall survival in affected patients [3,[10][11][12][13][14][15]. MZBL composed of CD20+ B cells is amenable to treatment with rituximab in conjunction with RT [10]. Additionally, the use of RT alone has also been shown to improve overall survival, without significant differences to outcomes of chemotherapy alone or combined chemo-RT [12].…”

Primary central nervous system marginal zone lymphoma

“…Unusual presentations include visual loss following bilateral progressive cranial nerve dysfunction; hearing loss due to diffuse pachymeningeal thickening, which mimics CNS infection or vasculitis 14 ; prolonged seizures and neurological decline in the case of diffuse pial enhancement have been also reported. 30 More rarely, acute subdural haematoma-like 18,[31][32][33][34][35][36][37][38] or stroke-like 39 presentation can occur.…”

“…56,68 Cytological examination, flow cytometry-based immunophenotyping and molecular analysis of IGH rearrangement on CSF may be helpful in the differential diagnosis with non-neoplastic morphological mimickers. 18,56 Genetic data and mutational landscape of PCNSMZL are rare and limited to dural-MZLs. The most common recurrent abnormality involves TNFAIP3 (A20), a negative regulator of the NF-kB signalling.…”

Different facets of intracranial central nervous system lymphoma and its imaging mimics

“…[ 16 ] The key diagnostic features include diffusely enhancing single or multiple extra-axial masses, [ 1 , 2 , 6 ] which often mimic meningiomas or subdural hematoma. [ 18 ] They are most commonly found in cerebral convexities, also in falx, tentorium, sellar/parasellar regions, or spine. [ 17 ] Imaging features again follow its hypercellular nature, being iso- to hyper-attenuating on CT, iso- to hypo- intense on T2-weighted images with restricted diffusion [ Figure 9 ].…”