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Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
The lymph system is part of the immune system and is made up of the following:
Anatomy of the lymph system, showing the lymph vessels and lymph organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymph vessels and into the lymph nodes where the lymphocytes destroy harmful substances. The lymph enters the blood through a large vein near the heart.
Because lymph tissue is found throughout the body, childhood non-Hodgkin lymphoma can begin in almost any part of the body. Cancer can spread to the liver and many other organs and tissues.
Non-Hodgkin lymphoma can occur in both adults and children. Treatment for children is different than treatment for adults. (See the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information on treatment in adults.)
There are four major types of childhood non-Hodgkin lymphoma.
The specific type of lymphoma is determined by how the cells look under a microscope. The 4 major types of childhood non-Hodgkin lymphoma are:
There are other types of lymphoma that occur in children. These include the following:
Possible signs of childhood non-Hodgkin lymphoma include breathing problems and swollen lymph nodes.
These and other symptoms may be caused by childhood non-Hodgkin lymphoma. Other conditions may cause the same symptoms. Check with a doctor if your child has any of the following problems:
Tests that examine the body and lymph system are used to detect (find) and diagnose childhood non-Hodgkin lymphoma.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
After childhood non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Some of the tests that are used to diagnose childhood non-Hodgkin lymphoma are also used to stage the disease. The following tests and procedures may be used in the staging process:
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
The following stages are used for childhood non-Hodgkin lymphoma:
Stage I
Stage I childhood non-Hodgkin lymphoma. Cancer is found in one group of lymph nodes or one area outside the lymph nodes, but no cancer is found in the abdomen or mediastinum (area between the lungs).
In stage I childhood non-Hodgkin lymphoma, cancer is found:
No cancer is found in the abdomen or mediastinum (area between the lungs).
Stage II
Stage II childhood non-Hodgkin lymphoma. Cancer is found in one area outside the lymph nodes and in nearby lymph nodes (a); or in two or more areas above (b) or below (c) the diaphragm; or cancer started in the stomach, appendix, or intestines (d) and can be removed by surgery.
In stage II childhood non-Hodgkin lymphoma, cancer is found:
Stage III
Stage III childhood non-Hodgkin lymphoma. Cancer is found in at least one area above and below the diaphragm (a); or cancer started in the chest (b); or cancer started in the abdomen and spread throughout the abdomen (c); or in the area around the spine (not shown).
In stage III childhood non-Hodgkin lymphoma, cancer is found:
Stage IV
Stage IV childhood non-Hodgkin lymphoma. Cancer is found in the bone marrow, brain, or cerebrospinal fluid (CSF). Cancer may also be found in other parts of the body.
In stage IV childhood non-Hodgkin lymphoma, cancer is found in the bone marrow, brain, or cerebrospinal fluid. Cancer may also be found in other parts of the body.
Childhood non-Hodgkin lymphoma is also described as low-stage or high-stage.
Treatment for childhood non-Hodgkin lymphoma is based on whether the cancer is low-stage or high-stage. Low-stage lymphoma has not spread beyond the area in which it began. High-stage lymphoma has spread beyond the area in which it began. Stage I and stage II are usually considered low-stage. Stage III and stage IV are usually considered high-stage.
Recurrent childhood non-Hodgkin lymphoma is cancer that has recurred (come back) after it has been treated. Childhood non-Hodgkin lymphoma may come back in the lymph system or in other parts of the body.
There are different types of treatment for children with non-Hodgkin lymphoma.
Different types of treatment are available for children with non-Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with non-Hodgkin lymphoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with non-Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists:
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)
Four types of standard treatment are used:
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas. Intrathecal chemotherapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain. When used to prevent cancer from spreading to the brain, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. Intrathecal chemotherapy is given in addition to chemotherapy by mouth or vein. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Intrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed.
Combination chemotherapy is treatment using 2 or more anticancer drugs.
See Drugs Approved for Non-Hodgkin Lymphoma for more information.
Radiation therapy (in certain patients)
Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
High-dose chemotherapy with stem cell transplant
This treatment is a way of giving high doses of chemotherapy and then replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
See Drugs Approved for Non-Hodgkin Lymphoma for more information.
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| Stem cell transplant (Step 1). Blood is taken from a vein in the arm of the donor. The patient or another person may be the donor. The blood flows through a machine that removes the stem cells. Then the blood is returned to the donor through a vein in the other arm. | Stem cell transplant (Step 2). The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown). | Stem cell transplant (Step 3). The patient receives stem cells through a catheter placed into a blood vessel in the chest. |
Stem cell transplant (Step 1). Blood is taken from a vein in the arm of the donor. The patient or another person may be the donor. The blood flows through a machine that removes the stem cells. Then the blood is returned to the donor through a vein in the other arm.
Stem cell transplant (Step 2). The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown).
Stem cell transplant (Step 3). The patient receives stem cells through a catheter placed into a blood vessel in the chest.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodies and tyrosine kinase inhibitors are two types of targeted therapy being studied in the treatment of childhood non-Hodgkin lymphoma.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is used to treat recurrent childhood non-Hodgkin lymphoma.
Tyrosine kinase inhibitors (TKIs) block signals that tumors need to grow. Some TKIs also keep tumors from growing by preventing the growth of new blood vessels to the tumors. Other types of kinase inhibitors, such as crizotinib and temsirolimus, are being studied for childhood non-Hodgkin lymphoma.
See Drugs Approved for Non-Hodgkin Lymphoma for more information.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your child's doctor for clinical trials that are not listed here but may be right for your child.
Low-stage Childhood Non-Hodgkin Lymphoma
Treatment of low-stage (stage I or II) non-Hodgkin lymphoma in children and adolescents may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I childhood large cell lymphoma, stage I childhood small noncleaved cell lymphoma, stage I childhood lymphoblastic lymphoma, stage I childhood anaplastic large cell lymphoma, stage II childhood large cell lymphoma, stage II childhood small noncleaved cell lymphoma, stage II childhood lymphoblastic lymphoma and stage II childhood anaplastic large cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
High-stage Childhood B-cell Non-Hodgkin Lymphoma
Treatment for high-stage (stage III or IV) B-cell (Burkitt and Burkitt-like) non-Hodgkin lymphoma in children and adolescents may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III childhood large cell lymphoma, stage III childhood small noncleaved cell lymphoma, stage IV childhood large cell lymphoma and stage IV childhood small noncleaved cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
High-stage Childhood Lymphoblastic Lymphoma
Treatment of high-stage (stage III or IV) lymphoblastic lymphoma in children and adolescents may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III childhood lymphoblastic lymphoma and stage IV childhood lymphoblastic lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
High-stage Childhood Anaplastic Large-cell Lymphoma
Treatment of high-stage (stage III or IV) anaplastic large-cell lymphoma in children and adolescents may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III childhood anaplastic large cell lymphoma and stage IV childhood anaplastic large cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent Childhood Non-Hodgkin Lymphoma
There is no standard treatment for patients with recurrent childhood non-Hodgkin lymphoma.
All patients with recurrent childhood non-Hodgkin lymphoma should be considered for clinical trials of new treatments.
Burkitt lymphoma and diffuse large B-cell lymphoma
Treatment options for recurrent Burkitt lymphoma and diffuse large B-cell lymphoma include:
Lymphoblastic lymphoma
Treatment options for recurrent lymphoblastic lymphoma include:
Anaplastic large-cell lymphoma
Treatment options for recurrent anaplastic large cell lymphoma include:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood non-Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Lymphoproliferative Disease Associated with a Weakened Immune System
Treatment of lymphoproliferative disease in children and adolescents with weakened immune systems may include the following:
For more information from the National Cancer Institute about childhood non-Hodgkin lymphoma, see the following:
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
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PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
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PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
Last Revised: 2012-11-26
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