What Is Childhood 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, 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.
How We Treat Childhood Primary Mediastinal B-cell Lymphoma
Children and teens with primary mediastinal B-cell lymphoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Childhood Lymphoma Program in our Childhood Hematologic Malignancy Center. Dana-Farber/Boston Children's offers internationally renowned care for children with cancers of the blood and immune system.
Dana-Farber/Boston Children's also offers a wide array of support services and programs for pediatric patients and their families during and after cancer treatment.
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 necessary.
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.
Treating Relapsed or Refractory Primary Mediastinal B-cell Lymphoma
For childhood 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.
Learn about treatment for childhood Burkitt lymphoma.
If radiation therapy was not part of the initial treatment, it may be recommended to treat refractory primary mediastinal B-cell lymphoma.
Long-term Follow-up for Children with Primary Mediastinal B-cell Lymphoma
Childhood cancer was once considered to be invariably fatal, but today, the majority of children diagnosed with cancer can expect to be long-term survivors.
Along with survivorship come numerous complex issues: the long-term effects of treatment and the risk of second cancers, as well as social and psychological concerns. For these reasons, survivors of childhood primary mediastinal B-cell lymphoma should receive regular follow-up monitoring and care.
Since 1993, physicians, nurses, researchers, and psychologists in our pediatric cancer survivorship programs at the David B. Perini Jr. Quality of Life Clinic at Dana-Farber have helped thousands of childhood cancer survivors, treated at Dana-Farber/Boston Children's and at other hospitals in New England and elsewhere, to manage these long-term challenges of surviving cancer.
Childhood Primary Mediastinal B-cell Lymphoma Treatment Team
Dana-Farber/Boston-Children's patients have access to the broadest set of pediatric hematologic and oncologic expertise available. The breadth of our expertise allows us to assemble a team of specialists to meet the specific needs of your child.