Primary mediastinal B-cell lymphoma: treatment and therapeutic targets.
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.
Rodríguez
José
J
Medical Oncology Department, Hospital Universitario Gregorio Maranon, Madrid, Spain.
Gutiérrez
Antonio
A
Piris
Miguel
M
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*
D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor