Extranodal marginal zone lymphoma of the dura mater with IgH/MALT1 translocation and review of literature

Abstract

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma involving brain, intraocular structures and spinal cord, without evidence of systemic disease. The majority of PCNSLs are diffuse large B-cell type. We encountered a rare case of primary dural marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) with extension into the brain in a 59-year-old man. A magnetic resonance imaging scan showed a 22-mm tumor located in the left posterior temporal lobe extending from the dura. Histopathology revealed a lymphoplasmacytic infiltration of the dura and the brain parenchyma in a perivascular pattern. Immunohistochemical and in situ hybridization studies showed a B-cell phenotype with kappa light chain restriction. Fluorescent in situ hybridization study showed a t(14;18)(q32;q21) with immunoglobulin heavy-chain/MALT1 fusion. The molecular study for immunoglobulin heavy-chain gene rearrangement by polymerase chain reaction showed a clonal gene rearrangement.

Marginal zone lymphomas involving meningeal dura: possible link to IgG4-related diseases

“…Although the number of cases studied for genetic alterations is small and precludes generalization, trisomy 3 remains the most frequent aberration as also reported by Tu et al 7 Although there is a single report documenting a dural marginal zone lymphoma with IGH/MALT-1 translocation, 8 our study failed to detect MALT-or IGH-related translocations, in concurrence with the findings of Tu et al 7 In addition, none of these genetic alterations were detected in the IgG4-positive dural marginal zone lymphomas tested, (Table 3) suggesting that dural marginal zone lymphomas may be distinct from orbital MALT lymphomas, which harbor IGH translocations in a significant proportion of cases.…”

“…[5][6][7] Among the recurrent cytogenetic abnormalities described in mucosa-associated lymphoid tissue (MALT) lymphomas, only trisomy 3 was detected in dural marginal zone lymphomas in a large series, 7 whereas trisomy 18 and translocations, such as t(11;18)(q21;q21) and t(14;18)(q32;q21) involving API2-MALT and immunoglobulin heavy-chain-joining region (IGH)-MALT, respectively, were not detected 7 with the exception of one report describing presence of IGH-MALT1 at this location. 8 This finding is not surprising, given that MALT lymphomas have site-specific differences in the frequencies of the various cytogenetic abnormalities; lung MALT lymphomas (and to a lesser extent gastric MALT lymphomas) frequently harbor the API2-MALT, 9 whereas orbital MALTs 10 are known to often harbor translocations involving IGH@. 11 MALT lymphomas arise in the background of chronic inflammation and autoimmune conditions with chronic Helicobacter pylori gastritis or Hashimoto's thyroiditis, representing quintessential examples for harbingers of marginal zone lymphoma in their respective locations.…”

Primary Central Nervous System Mucosa-Associated Lymphoid Tissue Lymphoma-Case Report-

“…18) Only small numbers of CNS MALT lymphomas have been reported. 2,6,7,[11][12][13][15][16][17][18][19]21,22,[24][25][26][27][28]31,33) Most cases of CNS MALT lymphomas occur in middle-aged women in contrast to primary CNS lymphoma which has a slight male predilection, although no specific factors related to sex have been identified. Most patients with CNS MALT lymphoma in the cranium present with insidious onset of headaches, seizure, focal sensory disturbances, and CT/MR imaging findings of well-defined, intracranial, dural masses, which are diagnosed clinically as meningioma.…”

“…10) Immunophenotyping shows the cells express B-cell-associated antigen such as CD20 and CD79a with no expression of CD5, CD10, CD23, and cyclinD1. 7) MALT lymphomas are characterized by a cytological composition that can range from small lymphocytic to plasmacytoid to marginal zone type cells. These histological findings are also observed in small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) and lymphoplasmacytoid lymphoma (LPL).…”

“…MALT lymphoma originating from meningeal tissue is rare, and only a handful of cases and small series have been described. 2,6,7,[11][12][13][15][16][17][18][19]21,22,[24][25][26][27][28]31,33) This subtype is distinct from other primary CNS lymphoma or metastatic CNS lymphoma that can have poor outcomes. Therefore, recognition of this rare subtype of CNS lymphoma is important.…”