title: Nodal marginal zone lymphoma: a heterogeneous tumor: a comprehensive analysis of a series of 27 cases.
abstract: We have reviewed the clinical, morphologic, immunophenotypical, and molecular features of a series of 27 cases of nodal marginal zone lymphoma with the aim of defining this entity more precisely. The series was characterized by a relatively favorable clinical course, with a low clinical stage at diagnosis (59% patients with clinical stage I-II) and a 5-year overall survival probability of 79%. However, the disease persisted in a relatively large fraction of the patients, thus yielding a 5-year failure-free survival probability of 22%. Molecular and immunohistochemical analyses of the series revealed heterogeneity in the frequency of IgV(H) somatic mutation and in the expression of IgD, CD43, MUM1, and CD38. Apart from the absence of nuclear Bcl10, no clear distinction could be made from the expression profiling of other B-cell lymphomas claimed to be derived from marginal zone B cells. Additionally, the immunophenotype of the tumoral cells in all cases but one differed from that described in monocytoid B cells. It was characterized by a Bcl2-, p21+, cyclin E+ profile. The analysis of apoptosis-regulator proteins disclosed abnormalities in the expression of survivin and active caspase 3, which could partially explain the abnormal regulation of apoptosis observed in these tumors. Molecular and immunohistochemical data obtained in this study strongly imply that there is significant heterogeneity among the cases included in the category termed nodal marginal zone lymphoma.
authors.affiliation: Lymphoma Group, Molecular Pathology Program, Centro Nacional de Investigaciones Oncológicas, Madrid, Spain. fcamacho@cnio.es
authors.forename: Francisca I
authors.initials: FI
authors.lastname: Camacho
authors.forename: Patricia
authors.initials: P
authors.lastname: Algara
authors.forename: Manuela
authors.initials: M
authors.lastname: Mollejo
authors.forename: Juan F
authors.initials: JF
authors.lastname: García
authors.forename: Carlos
authors.initials: C
authors.lastname: Montalbán
authors.forename: Nerea
authors.initials: N
authors.lastname: Martínez
authors.forename: Margarita
authors.initials: M
authors.lastname: Sánchez-Beato
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Piris
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000032:analysis; D005260:Female; D005803:Genes, Immunoglobulin; D006801:Humans; D007150:Immunohistochemistry; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D011379:Prognosis; D016019:Survival Analysis
chemical_list: D015415:Biomarkers