Primary mediastinal large B-cell lymphoma.
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.
Department of Internal Medicine, Division of Hematology-Oncology, University of Nebraska Medical Center, United States. Electronic address: vijaya.bhatt@unmc.edu.
Vijaya Raj
VR
Bhatt
Department of Internal Medicine, Creighton University Medical Center, United States.
Rajesh
R
Mourya
Department of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY, United States.
Runa
R
Shrestha
Department of Internal Medicine, Division of Hematology-Oncology, University of Nebraska Medical Center, United States.
James O
JO
Armitage
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D049268:Positron-Emission Tomography; D016879:Salvage Therapy
Chemotherapy; Mediastinal radiation; Positron emission tomography; Primary mediastinal large B-cell lymphoma; Rituximab