General Information About Childhood Non-Hodgkin Lymphoma

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:

• Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumors.
• Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
• Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes grow along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.
• Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach.
• Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
• Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes.
• Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets.

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:

• B-cell non-Hodgkin lymphoma (Burkitt and Burkitt-like lymphoma) and Burkitt leukemia.
• Diffuse large B-cell lymphoma.
• Lymphoblastic lymphoma.
• Anaplastic large cell lymphoma.

There are other types of lymphoma that occur in children. These include the following:

• Lymphoproliferative disease associated with a weakened immune system.
• Rare non-Hodgkin lymphomas that are more common in adults than in children.

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. A doctor should be consulted if any of the following problems occur:

• Shortness of breath.
• Trouble breathing.
• Wheezing.
• High-pitched breathing sounds.
• Swelling of the head or neck.
• Trouble swallowing.
• Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin.
• Fever for no known reason.
• Weight loss for no known reason.
• Night sweats.

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:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. One of the following types of biopsies may be done:
  • Excisional biopsy: The removal of an entire lymph node or lump of tissue.
  • Incisional biopsy: The removal of part of a lump, lymph node, or sample of tissue.
  • Core biopsy: The removal of tissue or part of a lymph node using a wide needle.
  • Fine-needle aspiration (FNA) biopsy: The removal of tissue or part of a lymph node using a thin needle.
  • Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone.Certain factors affect prognosis (chance of recovery) and treatment options.

    The prognosis (chance of recovery) and treatment options depend on:

    • The stage of the cancer.
    • The number of places outside of the lymph nodes to which the cancer has spread.
    • The type of lymphoma.
    • The patient’s general health.

    Stages of Childhood Non-Hodgkin Lymphoma

    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:

    • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
    • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
      • The number of red blood cells, white blood cells, and platelets.
      • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
      • The portion of the sample made up of red blood cells.
      The following stages are used for childhood non-Hodgkin lymphoma:Stage I

      In stage I childhood non-Hodgkin lymphoma, cancer is found in a single area or lymph node outside of the abdomen or chest.

      Stage II

      In stage II childhood non-Hodgkin lymphoma, cancer is found:

      • in only one area and in the lymph nodes around it; or
      • in 2 or more areas or lymph nodes on one side of the diaphragm (the thin muscle under the lungs that divides the chest and abdominal cavity and helps with breathing); or
      • to have started in the stomach or intestines and has been completely removed by surgery, and lymph nodes in the area may or may not have cancer.
      Stage III

      In stage III childhood non-Hodgkin lymphoma, cancer is found:

      • in areas or lymph nodes on both sides of the diaphragm; or
      • to have started in the chest; or
      • in more than one place in the abdomen; or
      • in the area around the spine.
      Stage IV

      In stage IV childhood non-Hodgkin lymphoma, cancer is found in the bone marrow, brain, or spinal cord. Cancer may also be found in other parts of the body.

      Childhood non-Hodgkin lymphoma is also described as localized or disseminated.

      Treatment for childhood non-Hodgkin lymphoma is based on whether the cancer is localized or disseminated. Localized lymphoma has not spread beyond the area in which it began. Disseminated lymphoma has spread beyond the area in which it began. Stage I and stage II are usually considered localized. Stage III and stage IV are usually considered disseminated.

      Recurrent Childhood Non-Hodgkin Lymphoma

      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.

      Treatment Option Overview

      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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

      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 pediatric doctors who are experts in treating children with non-Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists:

      • Radiation oncologist.
      • Pediatric hematologist.
      • Pediatric surgeon.
      • Pediatric nurse specialist.
      • Rehabilitation specialist.
      • Psychologist.
      • Social worker.
      Some cancer treatments cause side effects months or years after treatment has ended.

      Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include:

      • Physical problems.
      • Changes in mood, feelings, thinking, learning, or memory.
      • Second cancers (new types of cancer).

      Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

      Three 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 spinal column (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 spread to the brain, it is called CNS prophylaxis. The way the chemotherapy is given depends on the type and stage of the cancer being treated.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. When radiation therapy is used to prevent spread to the brain, it is called CNS prophylaxis. 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.

      New types of treatment are being tested in clinical trials. These include the following:Monoclonal antibody therapy

      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.

      This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.

      Treatment Options for Childhood Non-Hodgkin Lymphoma

      Localized Non-Hodgkin Lymphoma in Children and Adolescents

      Standard treatment of localized non-Hodgkin lymphoma in children and adolescents is usually surgery followed by combination chemotherapy.

      New treatments are being studied in clinical trials for localized non-Hodgkin lymphoma in children and adolescents. Information about ongoing clinical trials is available from the NCI Web site.

      Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry 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.

      Disseminated Childhood B-cell Non-Hodgkin Lymphoma

      Standard treatment for disseminated B-cell (Burkitt and Burkitt-like) non-Hodgkin lymphoma in children and adolescents is usually combination chemotherapy with CNS prophylaxis (intrathecal chemotherapy).

      Monoclonal antibody therapy combined with chemotherapy is being studied in clinical trials for disseminated B-cell non-Hodgkin lymphoma in children and adolescents. Information about this and other ongoing clinical trials is available from the NCI Web site.

      Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry 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.

      Disseminated Childhood Lymphoblastic Lymphoma

      Standard treatment of disseminated lymphoblastic lymphoma in children and adolescents is usually combination chemotherapy with or without radiation therapy to the brain.

      New combinations of chemotherapy, with and without CNS prophylaxis (intrathecal chemotherapy), are being studied in clinical trials for disseminated lymphoblastic lymphoma in children and adolescents. Information about these and other ongoing clinical trials is available from the NCI Web site.

      Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III childhood lymphoblastic lymphoma and stage IV childhood lymphoblastic lymphoma.

      Disseminated Childhood Anaplastic Large-cell Lymphoma

      Standard treatment of disseminated anaplastic large-cell lymphoma in children and adolescents is usually combination chemotherapy with or without CNS prophylaxis (intrathecal chemotherapy).

      New combinations of chemotherapy are being studied in clinical trials for disseminated anaplastic large-cell lymphoma in children and adolescents. Information about these and other ongoing clinical trials is available from the NCI Web site.

      Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III childhood anaplastic large cell lymphoma and stage IV childhood anaplastic large cell lymphoma.

      Recurrent Childhood Non-Hodgkin Lymphoma

      Standard treatment of recurrent childhood non-Hodgkin lymphoma in children and adolescents may include the following:

      • High-dose chemotherapy with stem cell transplant.
      • Combination chemotherapy.

      Information about ongoing clinical trials is available from the NCI Web site.

      Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood non-Hodgkin lymphoma.

      Lymphoproliferative Disease Associated with a Weakened Immune System

      Standard treatment of lymphoproliferative disease in children and adolescents with weakened immune systems may include the following:

      • Surgery with or without radiation therapy.
      • Combination chemotherapy.

      Some of the treatments being studied in clinical trials include the following:

      • Monoclonal antibody therapy combined with chemotherapy.
      • Stem cell transplant followed by donor lymphocyte infusion or an infusion of T-cell lymphocytes that have been treated in the laboratory.

      Information about these and other ongoing clinical trials is available from the NCI Web site.

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      Changes to This Summary (02/22/2008)

      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.

      Images were added to this summary.