Childhood non-Hodgkin lymphoma is a type of cancer that forms in the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system 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.
Non-Hodgkin lymphoma can begin in B lymphocytes, T lymphocytes, or natural killer cells. Lymphocytes can also be found in the blood and collect in the lymph nodes, spleen, and thymus.
Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin.
Non-Hodgkin lymphoma can occur in both adults and children. Treatment for children is different than treatment for adults. See the following PDQ summaries for information about treatment of non-Hodgkin lymphoma in adults:
Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of childhood non-Hodgkin lymphoma. See the PDQ summary on Childhood Hodgkin Lymphoma Treatment for information about childhood Hodgkin lymphoma.There are three major types of childhood non-Hodgkin lymphoma.
The type of lymphoma is determined by how the cells look under a microscope. The three major types of childhood non-Hodgkin lymphoma are:Mature B-cell non-Hodgkin lymphoma
Mature B-cell non-Hodgkin lymphomas include:
Both Burkitt leukemia and Burkitt lymphoma have been linked to infection with the Epstein-Barr virus (EBV), although EBV infection is more likely to occur in patients in Africa than in the United States. Burkitt and Burkitt-like lymphoma/leukemia are diagnosed when a sample of tissue is checked and a certain change to the c-myc gene is found.
Lymphoblastic lymphoma is a type of lymphoma that mainly affects T-cell lymphocytes. It usually forms in the mediastinum (the area behind the breastbone). This causes trouble breathing, wheezing, trouble swallowing, or swelling of the head and neck. It may spread to lymph nodes, bone, bone marrow, skin, the CNS, abdominal organs, and other areas. Lymphoblastic lymphoma is a lot like acute lymphoblastic leukemia (ALL).Anaplastic large cell lymphoma
Anaplastic large cell lymphoma is a type of lymphoma that mainly affects T-cell lymphocytes. It usually forms in the lymph nodes, skin, or bone, and sometimes forms in the gastrointestinal tract, lung, tissue that covers the lungs, and muscle. Patients with anaplastic large cell lymphoma have a receptor, called CD30, on the surface of their T cells. In many children, anaplastic large cell lymphoma is marked by changes in the ALK gene that makes a protein called anaplastic lymphoma kinase. A pathologist checks for these cell and gene changes to help diagnose anaplastic large cell lymphoma.Some types of non-Hodgkin lymphoma are rare in children.
Some types of childhood non-Hodgkin lymphoma are less common. These include:
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.
Possible risk factors for childhood non-Hodgkin lymphoma include the following:
If lymphoma or lymphoproliferative disease is linked to a weakened immune system from certain inherited diseases, HIV infection, a transplant or medicines given after a transplant, the condition is called lymphoproliferative disease associated with immunodeficiency. The different types of lymphoproliferative disease associated with immunodeficiency include:
These and other signs may be caused by childhood non-Hodgkin lymphoma or by other conditions. Check with a doctor if your child has any of the following:
The following tests and procedures may be used:
Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
Lumbar puncture. A patient lies in a curled position on a table. After a small area on the lower back is numbed, a spinal needle (a long, thin needle) is inserted into the lower part of the spinal column to remove cerebrospinal fluid (CSF, shown in blue). The fluid may be sent to a laboratory for testing.
Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of non-Hodgkin lymphoma, biopsy samples should be checked by a pathologist who has experience in diagnosing childhood non-Hodgkin lymphoma.
One of the following types of biopsies may be done:
The procedure used to remove the sample of tissue depends on where the tumor is in the body:
Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the child’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
If cancer is found, the following tests may be done to study the cancer cells:
The prognosis (chance of recovery) and treatment options depend on:
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 results of tests and procedures used to diagnose non-Hodgkin lymphoma may also be used for staging. See the General Information section for a description of these tests and procedures. 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.
The following procedure also may be used to determine the stage:
Cancer can spread through tissue, the lymph system, and the blood:
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 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 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.
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.
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.
Taking part in a clinical trial should be considered for all children with non-Hodgkin lymphoma. 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 who are experts 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:
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.)Six 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. Combination chemotherapy is treatment using two or more anticancer drugs.
The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Intrathecal chemotherapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain. When used to lessen the chance cancer will spread to the brain, it is called CNS prophylaxis. Intrathecal chemotherapy is given in addition to chemotherapy by mouth or vein. Higher than usual doses of chemotherapy may also be used as CNS prophylaxis.
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.
See Drugs Approved for Non-Hodgkin Lymphoma for more information.Radiation therapy
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:
The way the radiation therapy is given depends on the type of non-Hodgkin lymphoma being treated. External radiation therapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain and spinal cord. Internal radiation therapy is not used to treat non-Hodgkin lymphoma.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.
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. (Step 2): The patient receives chemotherapy to kill blood-forming cells. The patient may receive radiation therapy (not shown). (Step 3): The patient receives stem cells through a catheter placed into a blood vessel in the chest.
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, tyrosine kinase inhibitors, and immunotoxins are three types of targeted therapy being used or 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.
A bispecific monoclonal antibody is made up of two different monoclonal antibodies that bind to two different substances and kills cancer cells. Bispecific monoclonal antibody therapy is used in the treatment of Burkitt and Burkitt-like lymphoma/leukemia and diffuse large B-cell 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, are being studied for childhood non-Hodgkin lymphoma.
Immunotoxins can bind to cancer cells and kill them. Denileukin diftitox is an immunotoxin used to treat cutaneous T-cell lymphoma.
Targeted therapy is being studied for the treatment of childhood non-Hodgkin lymphoma that has recurred (come back).
See Drugs Approved for Non-Hodgkin Lymphoma for more information.Other drug therapy
Retinoids are drugs related to vitamin A. Retinoid therapy with bexarotene is used to treat several types of cutaneous T-cell lymphoma.
Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Steroid therapy is used to treat cutaneous T-cell lymphoma.Phototherapy
Phototherapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer in the skin, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Phototherapy is used in the treatment of cutaneous T-cell lymphoma.New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.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.
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.
Treatment options for newly diagnosed Burkitt and Burkitt-like lymphoma/leukemia may include:
Treatment options for recurrent Burkitt and Burkitt-like non-Hodgkin lymphoma/leukemia may include:
Treatment options for newly diagnosed diffuse large B-cell lymphoma may include:
Treatment options for recurrent diffuse large B-cell lymphoma may include:
Treatment options for newly diagnosed primary mediastinal B-cell lymphoma may include:
Treatment options for recurrent primary mediastinal B-cell lymphoma may include:
Lymphoblastic lymphoma may be classified as the same disease as acute lymphoblastic leukemia (ALL). Treatment options for lymphoblastic lymphoma may include:
Treatment options for recurrent lymphoblastic lymphoma may include:
Treatment options for anaplastic large cell lymphoma may include:
Treatment options for recurrent anaplastic large cell lymphoma may include:
Treatment options for lymphoproliferative disease in children and adolescents with weakened immune systems may include:
Treatment with highly active antiretroviral therapy or HAART (a combination of antiretroviral drugs) lowers the risk of non-Hodgkin lymphoma in patients infected with the human immunodeficiency virus (HIV).
Treatment options for HIV-related non-Hodgkin lymphoma (NHL) in children may include:
For treatment of recurrent disease, treatment options depend on the type of non-Hodgkin lymphoma.
Treatment options for post-transplant lymphoproliferative disease may include:
Treatment options for follicular lymphoma in children may include:
For children whose cancer has certain changes in the genes, treatment is similar to that given to adults with follicular lymphoma. See the Follicular Lymphoma section in the PDQ summary on Adult Non-Hodgkin Lymphoma for information.
Treatment options for marginal zone lymphoma (including mucosa-associated lymphoid tissue (MALT) lymphoma) in children may include:
Treatment options for primary CNS lymphoma in children may include:
Treatment options for peripheral T-cell lymphoma in children may include:
Treatment options for subcutaneous panniculitis-like cutaneous T-cell lymphoma in children may include:
Treatment options for cutaneous anaplastic large cell lymphoma may include:
In children, treatment options for mycosis fungoides may include:
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, see the following:
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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389294]
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Physicians version: CDR0000062808
Date last modified: 2018-04-04
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