It primarily affects younger patients in their 30's and 40's, and is more common in women than men. It closely resembles Hodgkin's lymphoma, and has a similar prognosis to similarly staged DLBC.
PMBCL is an aggressive lymphoma which often presents with symptoms related to its location. Breathing problems, shortness of breath, pleural effusions, superior vena cava syndrome, fevers. etc. On initial diagnosis it is almost always limited to the mediastinal mass, but if a relapse occurs it is likely to occur in the kidneys, liver, central nervous system.
PMBCL therapy and prognosis are the same for similarly staged patients with diffuse large b-cell lymphoma. The International Prognostic Index can assist with determining the overall prognosis.
Treatment consists of aggressive combination chemotherapy with the goal of curing the disease. In the event of a relapse a stem cell transplant is the preferred treatment option as is the case with other relapsed aggressive lymphomas. Historically CHOP or MACOP would be used, but with the introduction of Rituxan MACOP may not be as popular. R-CHOP is one option but some evidence is showing that Dose Adjusted R-EPOCH may have better results. (1)
The role of radiotherapy is uncertain. It was commonly used and often considered necessary due to the size of the tumours and the location. However radiation to the mediastinum carries risks to the heart, lungs and breast tissue. Here again, the addition of Rituxan to chemotherapy may eliminate the need for radiation due to the dramatic survival benefit Rituxan adds. (1) (2) (3) It is also shown that reserving radiation only for patients who only achieve a partial remission to initial therapy may be the best choice. (5) Those who achieve a complete remission from initial therapy do not benefit. The choice of whether or not to use radiotherapy should be made in consultation with the doctor and after a review of the post chemotherapy CT and/or PET scans.
Proton Beam Radiation instead of traditional radiation may be a better option. It appears deliver significantly less radiation to the major organs like the heart and lungs, when radiating a mediastinal mass. (4)