Central nervous system marginal zone B‐cell lymphoma of mucosa‐associated lymphoid tissue type involving the brain and spinal cord parenchyma

Abstract

We present a case of a 53-year-old HIV negative man with a 2-month history of progressive recent memory disturbance, gait disturbance and urinary incontinence. On MRI, an infiltrative tumor in the brain and spinal cord was noted. Subsequent positron emission tomography studies along with bone marrow biopsy and serum protein electrophoresis showed no evidence of systemic disease. Open brain biopsy results revealed a small lymphocytic infiltrate with scattered plasma cells in a predominantly perivascular growth pattern. The morphology was consistent with involvement by a low-grade B-cell lymphoma. Immunohistochemical findings showed CD20+, CD10-, CD5-, TdT-, EBV-encoded RNA in situ- and IgM-. The above findings were consistent with involvement by a non-dural extranodal marginal zone B-cell lymphoma (MZBCL) primary to the brain and spinal cord. This is a case report of a CNS MZBCL of mucosa-associated lymphoid tissue type involving the brain and spinal cord parenchyma.

Primary Intracranial Mucosa-Associated Lymphoid Tissue Lymphoma

“…Primary central nervous system (CNS) lymphomas represent 3-4% of primary brain tumours 5 . Large B-cell diffuse lymphomas are the most frequent subgroup, comprising 90% of all primary CNS lymphomas, most frequently seen in immune compromised patients as aggressive intraparenchymal tumours.…”

“…Large B-cell diffuse lymphomas are the most frequent subgroup, comprising 90% of all primary CNS lymphomas, most frequently seen in immune compromised patients as aggressive intraparenchymal tumours. These lesions very rarely involve the dura mater exclusively 5 . The marginal zone B-cell lymphomas, either primarily or secondarily involving the CNS, are very rare [5][6][7] .…”

“…These lesions very rarely involve the dura mater exclusively 5 . The marginal zone B-cell lymphomas, either primarily or secondarily involving the CNS, are very rare [5][6][7] . Whenever MALT lymphomas involve the CNS, they most commonly involve the dura mater in comparison to other CNS compartments 8 .…”

How we manage Bing–Neel syndrome

“…The sole identification of the MYD88 L265P mutation in the CSF, however, is not diagnostic for BNS, because this mutation can also be detected in a substantial proportion of patients with primary CNS lymphoma (Montesinos-Rongen et al, 2011;Gonzalez-Aguilar et al, 2012;Nakamura et al, 2016). The MYD88 L265P mutation can also be detected in a minority of patients with chronic lymphocytic leukaemia (CLL) or marginal zone lymphoma (MZL) and low-grade lymphoproliferative disorders, in which CNS involvement can also rarely develop (Jesionek-Kupnicka et al, 2013;Ueba et al, 2013;Rossi et al, 2014;Strati et al, 2016;Wanquet et al, 2016;Ayanambakkam et al, 2018).…”

The genetic landscape of dural marginal zone lymphomas

“…In contrast, the meninges are the most common primary (or secondary) site of involvement by low grade B-NHL [2]. The dura is a recognized but uncommon, primary site of occurrence of marginal zone lymphoma (MZL) and rare cases of intra-parenchymal MZLs, occurring in a perivascular location, have also been described [3, 4]. A female predilection has been documented for dural MZLs, and since they often present as solitary masses, they can mimic meningioma's clinically and on imaging studies [5–7].…”