Extranodal Marginal Zone Lymphoma of the Central Nervous System

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

“…Other diagnostic considerations include metastases, and inflammatory or infectious granulomatous disease such as Wegener's disease, Rosai‐Dorfman disease, syphilis, tuberculosis, or other secondary lymphoproliferative lesions . For example, dural MALT lymphomas have been associated with chronic inflammatory states such as hepatitis C and autoimmune diseases …”

“…A confirmatory diagnosis is obtained through pathological analysis via histology and/or cytology . Neoplastic cells spread diffusely and are found adjacent to the meningeal tissue.…”

“…It is a low‐grade, indolent disease, which classically lacks invasion of the brain parenchyma. MZBCL is further characterized by responsiveness to treatment and overall favorable prognosis …”

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).…”

Central nervous system involvement in primary bone marrow or splenic marginal zone lymphoma: Report of two cases and review of the literature

“…Table depicts the main types, histologies and patterns of disease localization of primary nervous system lymphomas …”