• Primary Mediastinal B-cell Lymphoma

    Primary mediastinal B-cell lymphoma (PMBL) is a cancer of mature B-lymphocytes. It is a form of non-Hodgkin lymphoma, which is a type of cancer that originates in cells of the immune system, called lymphocytes

    • Primary mediastinal B-cell lymphoma is a rare type of non-Hodgkin lymphoma and is most common in adolescents and young adults.
    • PMBL usually starts in the area of the thymus, in a part of the upper chest called the mediastinum.
    • Under the microscope, PMBL looks similar to both diffuse large B-cell lymphoma and to Hodgkin lymphoma.
    • Primary mediastinal B-cell lymphoma often presents with symptoms of cough, shortness of breath, or swelling of the head and neck, due to the tumor pressing on the windpipe and the large veins above the heart.
    • With current therapies, many children with primary mediastinal B-cell lymphoma are cured of the disease.

    Primary Mediastinal B-cell Lymphoma Treatment at Dana-Farber/Boston Children's

    Children with non-Hodgkin lymphoma are treated at Dana-Farber/Boston Children's through the Lymphoma Program in our Hematologic Malignancies Center. One of the top pediatric cancer centers worldwide, Dana-Farber/Boston Children’s combines the expertise of a premier cancer center – Dana-Farber Cancer Institute – and a world-class children’s hospital – Boston Children’s Hospital – to provide internationally-renowned care for children with cancers of the blood and immune system.

    What are the treatments for primary mediastinal B-cell lymphoma?

    The lymphoma specialists at Dana-Farber/Boston Children’s determine the best approach to treatment for each child's unique situation, based on the type of lymphoma, the extent of the disease, the patient’s medical condition, the patient and family’s preferences, and the most up-to-date medical knowledge about lymphoma therapies.

    Primary mediastinal B-cell lymphoma has a protein, called CD20, on the surface of the cancer cells. Rituximab is an immunotherapy drug that targets the CD20 protein. Modern treatments for PMBL include rituximab, along with chemotherapy. Traditionally, radiation therapy has been part of treatment for this disease, but with the addition of rituximab, radiation may no longer be a necessary part of the treatment.

    The most commonly used treatment regimen for primary mediastinal B-cell lymphoma, both in adolescents and in adults, is called dose-adjusted EPOCH-R. This chemotherapy regimen is given as a 96-hour long continuous infusion into an intravenous line, once every three weeks for six courses. The doses of the chemotherapy drugs are adjusted according to blood tests that are done in between treatment courses. The drugs that are included in this treatment are etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab. A drug called filgrastim is used to help the body’s white blood cells recover quickly after each course of chemotherapy.

    Relapsed or refractory primary mediastinal B-cell lymphoma

    For primary mediastinal B-cell lymphoma that does not respond to initial treatment (refractory) or that comes back after treatment (relapse), treatment recommendations are the same as for other relapsed or refractory mature B-cell lymphomas, such as Burkitt lymphoma.

    If radiation therapy was not part of the initial treatment, it may be recommended to treat refractory primary mediastinal B-cell lymphoma.