Primary CNS T-cell Lymphomas: A Clinical, Morphologic, Immunophenotypic, and Molecular Analysis.
Fiche publication
Date publication
décembre 2015
Journal
The American journal of surgical pathology
Auteurs
Membres identifiés du Cancéropôle Est :
Dr NICOLAE Alina
Tous les auteurs :
Menon MP, Nicolae A, Meeker H, Raffeld M, Xi L, Jegalian AG, Miller DC, Pittaluga S, Jaffe ES
Lien Pubmed
Résumé
Primary central nervous system (CNS) lymphomas are relatively rare with the most common subtype being diffuse large B-cell lymphoma. Primary CNS T-cell lymphomas (PCNSTL) account for <5% of CNS lymphomas. We report the clinical, morphologic, immunophenotypic, and molecular characteristics of 18 PCNSTLs. Fifteen cases were classified as peripheral T-cell lymphoma, not otherwise specified, 2 of which were of γδ T-cell derivation and 1 was TCR silent; there was 1 anaplastic large cell lymphoma, ALK-positive and 2 anaplastic large cell lymphoma, ALK-negative. Median age was 58.5 years (range, 21 to 81 y), with an M:F ratio of 11:7. Imaging results showed that 15 patients had supratentorial lesions. Regardless of subtype, necrosis and perivascular cuffing of tumor cells were frequently observed (11/18 cases). CD3 was positive in all cases but 1; 10/17 were CD8-positive, and 5/17 were CD4-positive. Most cases studied had a cytotoxic phenotype with expression of TIA1 (13/15) and granzyme-B (9/13). Polymerase chain reaction analysis of T-cell receptor γ rearrangement confirmed a T-cell clone in 14 cases with adequate DNA quality. Next-generation sequencing showed somatic mutations in 36% of cases studied; 2 had >1 mutation, and none showed overlapping mutations. These included mutations in DNMT3A, KRAS, JAK3, STAT3, STAT5B, GNB1, and TET2 genes, genes implicated previously in other T-cell neoplasms. The outcome was heterogenous; 2 patients are alive without disease, 4 are alive with disease, and 6 died of disease. In conclusion, PCNSTLs are histologically and genomically heterogenous with frequent phenotypic aberrancy and a cytotoxic phenotype in most cases.
Mots clés
Adult, Aged, Aged, 80 and over, Biomarkers, Tumor, analysis, Biopsy, Central Nervous System Neoplasms, chemistry, DNA Mutational Analysis, Female, Gene Rearrangement, delta-Chain T-Cell Antigen Receptor, Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor, Genetic Predisposition to Disease, Humans, Immunohistochemistry, Immunophenotyping, In Situ Hybridization, Lymphoma, T-Cell, chemistry, Male, Middle Aged, Molecular Diagnostic Techniques, Mutation, Phenotype, Polymerase Chain Reaction, Predictive Value of Tests, Prognosis, Receptors, Antigen, T-Cell, gamma-delta, genetics, T-Lymphocytes, chemistry, Time Factors, Young Adult
Référence
Am J Surg Pathol. 2015 Dec;39(12):1719-1729