• World Neurosurg · Feb 2020

    Review

    Primary Intra-Axial Diffuse Large B-Cell Lymphoma In Immunocompetent Patients: Clinical Impact Of Molecular Analysis And Histogenetic Evaluation.

    • Eduardo Cambruzzi.
    • Department of Pathology, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Department of Pathology, Complexo Hospitalar Santa Casa, Porto Alegre, RS, Brazil; Hospital N. Sra. da Conceição, Porto Alegre, RS, Brazil; Department of Pathology, Universidade Luterana do Brasil, Canoas, RS, Brazil; Instituto de Cardiologia, Fundação Universitária de Cardiologia, Porto Alegre, RS, Brazil. Electronic address: dudacambruzzi@yahoo.com.br.
    • World Neurosurg. 2020 Feb 1; 134: 215-220.

    AbstractPrimary central nervous system (CNS) diffuse large B-cell lymphoma (DLBCL) represents less than 1% of non-Hodgkin lymphomas and 2%-3% of brain tumors. Primary CNS DLBCL occurs sporadically in healthy patients. Tumor development and progression have been associated with reduced/absent expression of human leukocyte antigen class I and II proteins; increased expression of CXCR4, CXCL12, CXCR5, and CCR7; mutations of VH4/34, BCL6, MYC, and PAX5 genes; and rearrangement of immunoglobulin heavy and light chain genes. Generally, DLBCL is a single supratentorial lesion (60%-70%), and stereotactic biopsy and intraoperative examination are the main diagnostic methods. Distinctive histologic features are a diffuse growth pattern and angioinvasiveness. Most neoplastic cells resemble centroblasts and exhibit positive CD20, CD22, PAX5, CD79a, and MUM1 expression. The prognosis of primary CNS DLBCL is less favorable than that of nodal DLBCL, and DLBCL subtype, strong FOXP1 immunoreactivity, MYC and BCL2 overexpression, and BCL6 translocations are associated with poor prognosis.Copyright © 2019 Elsevier Inc. All rights reserved.

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