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This information is about a specific type of non-Hodgkin lymphoma (NHL), known as mediastinal large B-cell lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma.
Mediastinal large B-cell lymphoma is an rare form of NHL, accounting for about one in 50 of all cases. It is a type of diffuse large B-cell lymphoma and arises from a rare type of B-cell lymphocyte in the thymus gland, behind the breast bone. It can occur at any time from early adulthood to old age but is commoner in younger people between 25 and 40. It is twice as common in women as in men.
The causes of mediastinal large B-cell lymphoma are unknown. Mediastinal large B-cell lymphoma is not infectious and cannot be passed on to other people.
The word 'mediastinal' refers to the mediastinum, which is the part of the body deep inside the chest, between the lungs and behind the breast bone. This area contains the thymus gland and many lymph nodes. In mediastinal large B-cell lymphoma the lymphoma develops in the thymus gland and the lymph nodes of the mediastinum, and these become enlarged. This can lead to symptoms of breathlessness, a cough, or pain in the chest.
Sometimes there is swelling of the tissues of the neck, arms and face due to the swollen lymph nodes pressing on the veins in the chest. This swelling is known as superior vena cava obstruction (SVCO).
Other lymph nodes may also be affected, which can cause a painless swelling in the neck, armpit or groin. Some people have loss of appetite and tiredness.
Other symptoms may include night sweats, unexplained high temperatures and weight loss. These are known as B symptoms.
A diagnosis is made by removing an enlarged lymph node, or part of an affected organ, and examining the cells under the microscope (biopsy). You will be referred to a surgeon for this procedure. It is a small operation and may be done under a local or general anaesthetic. Sometimes a biopsy is taken during a CT scan, which helps the doctor to make sure that the biopsy is taken from the affected area.
If the lymph nodes within the mediastinum are the only ones affected, the biopsy is likely to be taken during a procedure known as a mediastinoscopy. This involves making a small cut in the chest, so that a thin, flexible tube with a camera attached can be inserted to see the lymph nodes and take a sample for biopsy.
Additional tests, including blood tests, x-rays, scans, and bone marrow samples, are then used to get more information about the type of lymphoma, and how far it has spread in the body. This information is used to help decide which treatment is most appropriate for you.
The stage of NHL describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver are involved.
The stage usually includes the letter A or B, which describes whether or not any B symptoms are present (eg stage 2B). Sometimes the lymphoma can start in areas outside the lymph nodes, and this is represented by the letter E, which stands for extranodal (eg stage 3AE).
For practical purposes, non-Hodgkin lymphomas are also divided into two groups. They are referred to as either low- or high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas tend to grow more quickly.
Mediastinal large B-cell lymphoma is a high-grade lymphoma, which means that it is faster-growing and needs prompt treatment.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is usually the main treatment for mediastinal large B-cell lymphoma. The type of chemotherapy that is most appropriate will depend on the extent of the lymphoma, and other factors such as your age and general health. Usually a combination of chemotherapy drugs is given into the veins by injections or drips. The treatment may last from 3–6 months.
A commonly used chemotherapy combination is the R-CHOP regimen. This includes the chemotherapy drugs vincristine, doxorubicin and cyclophosphamide, the steroid prednisolone, and the monoclonal antibody rituximab (Mabthera®).
Mediastinal large B-cell lymphoma usually responds well to chemotherapy. Some people will find that although the initial chemotherapy can get rid of all signs of the lymphoma, it can come back (recur) after a year or two and they will then need further treatment.
Radiotherapy is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. After the chemotherapy, radiotherapy is sometimes given to the mediastinal area.
High-dose chemotherapy with bone marrow or stem cell infusions has been used for some patients. This type of treatment involves very intensive chemotherapy and sometimes radiotherapy.
As side effects can be severe, some types of high-dose treatment are not given to people over the age of 45–50, while others can be given to people of up to 65 years old who are fit enough. The intensity of the treatment increases the risk of serious side effects for people over this age.
Steroids are drugs, which are often given with chemotherapy to help treat lymphomas. They also help you to feel better and can reduce feelings of sickness.
Monoclonal antibodies are drugs that recognise, target, and stick to particular proteins on the surface of cancer cells, and can stimulate the body's immune system to destroy these cells. This treatment is usually given with chemotherapy as part of a regimen called R-CHOP (see above).
Sometimes there is a risk of developing scar tissue in the affected lymph nodes after treatment for this type of lymphoma. The scar tissue can make it difficult to assess whether or not there are still lymphoma cells in the lymph glands. Sometimes a specific type of scan, known as a PET scan, may be used after completing treatment, to check whether any active lymphoma is still present.
Research into new treatments for mediastinal B-cell lymphoma is ongoing, and you might be invited by your doctor to take part in a clinical trial to compare a new treatment against the best available standard treatment. Your doctor must discuss the treatment with you, and have your informed consent before entering you into any clinical trial.
The need for practical and emotional support will of course be individual. For some people with mediastinal large B-cell lymphoma, life may seem largely unaffected; for others the diagnosis of cancer may be a cause of great fear and distress. If you would like to discuss the condition, its treatment, or the practical and emotional problems of living with mediastinal large B-cell lymphoma, please contact our cancer support service.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
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