title: Primary mediastinal B-cell lymphoma: treatment and therapeutic targets.
abstract: Primary mediastinal B-cell lymphoma (PMBCL) is a recognised subtype of diffuse large B-cell lymphoma according to the WHO classification that represents approximately 5% of aggressive lymphomas, and 2% of all cases of lymphomas. It presents with unique clinical, morphologic and immunophenotypic characteristics that define the disease. Retrospective studies have found that PMBCL patients have excellent survival rates with a distinct plateau and a trend to better outcome if treated with dose-intensified chemotherapy with MACOPB or VACOPB. In spite of the multiple molecular data known, generated on the pathogenesis of this tumour, treatment is still essentially based on a combination of chemo and immunotherapy. We take this opportunity for reviewing the recent biologic data provided by gene expression profiling of the tumour, for discussing new potential therapeutic targets.
authors.affiliation: Medical Oncology Department, Hospital Universitario Gregorio Maranon, Madrid, Spain.
authors.forename: José
authors.initials: J
authors.lastname: Rodríguez
authors.forename: Antonio
authors.initials: A
authors.lastname: Gutiérrez
authors.forename: Miguel
authors.initials: M
authors.lastname: Piris
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism; D002986:Clinical Trials as Topic; D020869:Gene Expression Profiling; D006801:Humans; D007167:Immunotherapy; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000378:metabolism / Q000628:therapy*; D008479:Mediastinal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000628:therapy*
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor