Primary mediastinal large B-cell lymphoma.
Primary mediastinal large B-cell lymphoma (PMLBCL) is a distinct clinical and biological disease from other types of DLBCL. It is more frequent in young female and constitutes 6%-10% of all DLBCL. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis. Molecular analysis shows it to be a distinct entity from other DLBCL. Rituximab CHOP/MACOP-B-like regimens followed by mediastinal radiotherapy (RT) were associated with a 5-years PFS of 75%-85%. More intensive regimens, as DA-EPOCH-R without mediastinal RT, have shown very promising results, but this therapeutic advance needs to be confirmed in further prospective trials. The role of consolidative mediastinal RT should be still better assess in prospective comparative studies. PET-CT scan is a powerful tool to define the real quality of response and it is hoped that future prospective trials may allow its role in the de-escalation of mediastinal RT.
Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy. Electronic address: martelli@bce.uniroma1.it.
Maurizio
M
Martelli
San Raffaele Scientific Institute, Milan, Italy.
Andrés
A
Ferreri
Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.
Alice
A
Di Rocco
Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.
Michela
M
Ansuinelli
Cancer Research UK Centre, University of Southampton, Southampton, United Kingdom.
Peter W M
PWM
Johnson
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008479:Mediastinal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D000072078:Positron Emission Tomography Computed Tomography; D011379:Prognosis
Chemotherapy; Consolidation radiotherapy; Histopathology; Immunotherapy; PET-CT scan; Primary mediastinal large B-cell lymphoma; Prognostic factors