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Lymphomas in Children and Adolescents
Cancer can be divided into leukaemia (mainly in blood and bone marrow) and solid tumours, where one or more lumps are the likely origin of disease. Lymphomas are solid tumours which can involve many different parts of the body and can present at different ages. Some lymphomas resemble leukaemias in their pattern of spread and are treated with leukaemia-type treatment protocols. Because the various types of lymphoma behave in different ways it is best to consider them under individual groupings.
Hodgkins Disease:
Hodgkins Disease usually presents as enlargment of the lymph glands which are
distributed in the neck, chest, armpits and groins. It is rare in young children and is
most likely to be seen in older children and adolescents. Patients may present with a lump
only, or they may notice fevers, night sweats, weight loss or itchiness of the skin.
To be sure of the diagnosis a piece of tissue needs to be biopsied by a surgeon which, after processing, is examined by a pathologist under a microscope. Both chemotherapy (drugs) and radiation therapy are effective in Hodgkins disease. In order to work out the best choice or combination of treatments for the individual it is necessary to do further tests to evaluate the extent of the disease beyond that which can be seen by the naked eye. These tests are referred to as "staging" and the result will allow your doctor to ascertain the stage (extent) and express it as a number. For example if the disease is not found anywhere outside the original lump it is called Stage 1. If two or more lumps are found close to each other it becomes Stage 11. If disease involves both the upper and lower half of the body it is Stage 111. If it has got into the blood stream and has spread to organs other than the lymph glands and their related structures it is called Stage IV. In addition, the letter A will be added if the patient has no major symptoms and the letter B is added if the symptoms are considered to be significant by your doctor. So one can be Stage 11A or 11B, for example.
The intensity and duration of treatment will to some extent depend on the stage. We will also be guided by the findings of the pathologist, who will recognise certain sub-types of this disease in the cells provided from the biopsy. The usual duration of therapy is in the range of six months. There are a number of drug combinations that can be effective and a specific outline of the drugs chosen for each patient will be provided, as well as a schedule of their frequency and method of administration. Both general side effects of chemotherapy and specific side effects of the drugs used in your schedule will also be provided in this booklet. If radiation therapy is included in your schedule, the relevant information will again be provided and you will have the opportunity to discuss these matters with our radiation therapy specialist.
Non-Hodgkins Lymphoma:
Non-Hodgkins Lymphomas (NHL) are more common than Hodgkins disease and, although they are
solid tumours, they have many features in common with acute lymphatic leukaemia (the
commonest cancer seen in childhood). The cancer cell of Non-Hodgkins Lymphoma is very
similar to the cell of acute lymphatic leukaemia but, unlike leukaemia, it frequently
presents as a lump in some part of the body. The most likely place that lump may present
is in the abdomen. It often produces no symptoms in the patient until it
becomes very large. In some cases, however, it may interfere with bowel or kidney
function.
Non-Hodgkins Lymphoma is very prone to spread via the blood stream and can do so very early in the course of the disease. Therefore, the initial tests we do (including scans and x-rays) delineate the lump (for example in the abdomen) and also look for possible spread away from the original lump. For this reason bone marrow tests, analysis of spinal fluid, and various total body scans may be required. If the disease has spread and cells are obtained from one of these sites through a needle, then it may not be necessary or appropriate to perform an operation before starting any other form of treatment. If, however, the disease is found to be localised to the abdomen with no spread beyond, then a specialist cancer surgeon may attempt removal of a majority of the cancer.
Chemotherapy is the main form of treatment used for Non-Hodgkins Lymphoma. The intensity and duration of treatment depends on the extent of the disease at presentation and the amount of tumour that can be removed by operation. Using all this information your doctor will design a treatment programme (schedule) appropriate to your situation. Radiation therapy is not usually included for this particular condition. If the disease has spread widely, and this unfortunately is the case in about two thirds of the patients, a very intensive course of chemotherapy may be required. This protocol may include drugs given by vein or as tablets and also given through the spinal fluid to protect the brain and spinal cord from cancer cells. It is clear that this is a very serious cancer that can spread quickly and become difficult to cure.
The next most common place where Non-Hodgkins Lymphoma in children and adolescents may present is in the centre of the chest, known as the mediastinum. In this region there is a gland called the thymus gland which is closely involved with the lymphatic system. This gland may enlarge without any symptoms but when it becomes very big it can obstruct the air tubes as well as the veins draining the head and neck. An x-ray of the chest will show the enlarged thymus gland and also possibly fluid in the lining of the lung. This form of Non-Hodgkins Lymphoma is also likely to spread to the bone marrow and spinal fluid. It may also spread to the lymph glands in the neck. Biopsy material can often be obtained from one of these three sites and the tests required for staging may not be as numerous as in other forms of lymphomas.
Fortunately, despite the high propensity to spread early, the "mediastinum type" Non-Hodgkins Lymphoma is especially responsive to chemotherapy regimens. The chemotherapy programme is very similar to the one used in some forms of leukaemia (acute lymphatic leukaemia) and will run for two years. Special treatment to protect the brain and spinal cord from the cancer cells is required and consists of injection of drugs into the spinal fluid and for some patients radiation to the brain may also be required. Radiation is not used for other parts of the body under normal circumstances. There is also no further role for surgical operations other than the original biopsy and rarely for complications.
Occasionally, Non-Hodgkins Lymphoma may present as a lump in a lymph gland, as a lump in the bone, in skin, thyroid gland, or the tonsils. The principles of diagnosis and treatment are similar as described for disease arising in the mediastinum (chest), but the choice for chemotherapy programme may differ and some aspects need to be highly individualised according to the particular findings in that patient. In some situations, when disease is localised and not advanced, the treatment does not need to be very intensive or prolonged. This does not apply to the mediastinum, because lymphoma arising there always requires two years of treatment.
During treatment, some tests need to be repeated at regular intervals to check that the disease is remaining under control. In addition, many tests are designed to recognise early signs of possible side-effects of treatment so that adjustments can be made if necessary. After treatment is finished checks continue and some tests may still be required but they are done less often as time goes by.
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