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Together is a new resource for anyone affected by pediatric cancer - patients and their parents, family members, and friends.
Learn MorePrimary mediastinal large B-cell lymphoma (PMBCL) is a rare type of non-Hodgkin lymphoma. Certain white blood cells, called thymic B lymphocytes (or thymic B cells), become abnormal. These cells of the immune system develop in a gland called the thymus. The thymus is located in a space between the lungs called the mediastinum.
PMBCL may spread to nearby organs, such as the lungs or the sac around the heart called the pericardium. The cancer may spread to lymph nodes and distant organs, such as the kidneys.
In pediatric cases, PMBCL is often seen in older teens.
Contact your doctor right away if you notice symptoms such as:
These symptoms may appear as the tumor grows. The tumor may push on a large vein called the superior vena cava. This vein carries blood from the head, neck, and arms back to the heart. When this happens, it can cause a group of serious problems known as superior vena cava syndrome. This condition needs treatment right away.
Risk factors may increase your child's chances of getting a disease. Little is known about the risk factors for PMBCL in children. For lymphoma, the risk factors we know so far are:
A cancer diagnosis requires tests and procedures. The doctor will do a physical exam, ask about medical history, and order tests such as:
If the biopsy shows cancer, the lab may do more tests, such as flow cytometry, immunophenotyping, and cytogenetic analysis. The doctor may order genetic tests to find any genetic changes in the cancer cells.
Primary mediastinal large B-cell lymphoma cells as seen through a microscope.
The care team will plan treatment based on the cancer stage. Some tumors grow rapidly. So, the doctor must stage the lymphoma as quickly as possible.
To find out the stage of the disease, doctors may order more tests such as:
The stage will tell how much cancer is present and if it has spread to other parts of the body.
The International Pediatric Non-Hodgkin Lymphoma Staging System divides the disease into 4 stages:
PMBCL can spread quickly, so rapid treatment is needed. Treatment depends on the following:
The main treatment for childhood PMBCL is chemotherapy and targeted therapy with rituximab. When possible, patients may have surgery to remove all or part of the tumor. The most common treatments used for PMBCL include DA-EPOCH-R or the Lymphome Malin de Burkitt (LMB) treatment, which includes chemotherapy with rituximab.
Targeted therapies work by acting on, or targeting, specific features of tumor cells. Scientists are testing drugs to see if they can block the signals that cause cancer cells to grow. Your care team may suggest targeted therapy, immunotherapy, bone marrow transplant, or a clinical trial.
Treatment can sometimes cause side effects. One side effect that may occur early in treatment is tumor lysis syndrome. This happens when cancer cells quickly die and break apart. The care team will watch your child closely for possible side effects. Talk to your child’s care team if you have questions.
A common chemotherapy plan for relapsed disease is RICE (rituximab, ifosfamide, carboplatin, etoposide) followed by a stem cell transplant. New targeted therapies are being studied. Some patients may be able to take part in clinical trials to test new treatments.
The 4-year overall survival rate for childhood PMBCL is about 85% in the United States.
Coping with a cancer diagnosis and treatment can be stressful. You may want to talk to a social worker, psychologist, or another mental health specialist.
Learn how to talk to your child about cancer.
After treatment, your child's doctor may use imaging tests and exams to watch for recurrence. Childhood cancer survivors should get follow-up care throughout their lives. Some treatments can cause late effects. These are health problems that happen months or years after the end of treatment.
After completing treatment, it is important to:
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Reviewed: June 2023