Primary mediastinal large B-cell lymphoma

Cancer Treat Rev. 2015 Jun;41(6):476-85. doi: 10.1016/j.ctrv.2015.04.006. Epub 2015 Apr 21.


The management of primary mediastinal large B-cell lymphoma (PMBCL) requires a balance between optimizing chances of cure and reducing risk of long-term toxicities. The combination of rituximab to cyclophosphamide, doxorubicin, vincristine and prednisone (RCHOP) followed by mediastinal radiation results in a plateau in progression-free survival after first few years of follow-up. In rituximab era, a negative positron emission tomography (PET) scan performed after the completion of immunochemotherapy has a high predictive value for durable remission. Consequently, end-of-therapy PET may be utilizable to avoid radiation without compromising survival. Additionally, intensified chemotherapy alone has shown excellent survival. PMBCL is frequently associated with amplification of programmed death ligand (PDL) 1/2 and constitutive activation of JAK-STAT and NFKB pathways; these may serve as promising therapeutic targets. Clinical trials that integrate novel therapies into upfront immunochemotherapy and utilize end-of-therapy PET scan to guide mediastinal radiation have potential to further enhance survival and prevent long-term toxicities.

Keywords: Chemotherapy; Mediastinal radiation; Positron emission tomography; Primary mediastinal large B-cell lymphoma; Rituximab.

Publication types

  • Review

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Female
  • Humans
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / mortality
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large B-Cell, Diffuse / therapy*
  • Male
  • Mediastinal Neoplasms / diagnosis
  • Mediastinal Neoplasms / mortality
  • Mediastinal Neoplasms / pathology
  • Mediastinal Neoplasms / therapy*
  • Neoplasm Staging
  • Positron-Emission Tomography
  • Salvage Therapy