Non Hodgkin's Lymphoma
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Primary Mediastinal Lymphoma

Primary mediastinal B-cell Lymphoma (PMBCL)
Primary mediastinal B-cell lymphoma is a subset of Diffuse Large B-cell lymphoma, which arises in the thymus, and presents as a mass in the mediastinum.

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.

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. The addition of Rituxan to chemotherapy appears to have negated the need for more intensive chemotherapy, and therefore R-CHOP may be preferred over regimens such as MACOP-B. Here is one study which shows the superiority of R-CHOP versus historical controls using only CHOP

Rituximab-CHOP (R-CHOP) and Radiotherapy (RT) for Primary Mediastinal Large B-Cell Lymphoma (PMLBCL)

The role of radiotherapy is uncertain. Some studies suggest there is an added benefit to radiation to the primary mass, while other studies show no such benefit. Here again, the addition of Rituxan to chemotherapy may eliminate the need for radiation due to the dramatic survival benefit Rituxan adds. 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.  Here are two studies regarding the use of radiation. The first indicates a benefit from adding radiation..

Primary mediastinal large B-cell lymphoma: Results of intensive chemotherapy regimens (MACOP-B/VACOP-B) plus involved field radiotherapy on 53 patients. A single institution experience

This next study shows that the addition of Rituxan eliminates the need for radiation.

Primary Mediastinal Large B-Cell Lymphoma (PMBL) Outcome May Be Significantly Improved by the Addition of Rituximab to Dose-Adjusted (DA)-EPOCH and Obviates the Need for Radiation: Results from a Prospective Study of 44 Patients

For more reading about PMBCL please read the following articles.

• A highly detailed look at primary mediastinal lymphoma from The Oncologist Journal
   

• Primary mediastinal lymphoma from e-medicine
   

• The American Society of Hematology annual convention collection of lymphoma abstracts. Search each of the past 3 years for the word Mediastinal

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