Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)—Patient Version - National Cancer Institute

Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Patient Version

General Information About Childhood Non-Hodgkin Lymphoma

KEY POINTS

Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
The main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma.
There are three major types of childhood non-Hodgkin lymphoma.
- Mature B-cell non-Hodgkin lymphoma
- Lymphoblastic lymphoma
- Anaplastic large cell lymphoma
Some types of non-Hodgkin lymphoma are rare in children.
Past treatment for cancer and having a weakened immune system affect the risk of having childhood non-Hodgkin lymphoma.
Signs of childhood non-Hodgkin lymphoma include breathing problems and swollen lymph nodes.
Tests that examine the body and lymph system are used to detect (find) and diagnose childhood non-Hodgkin lymphoma.
A biopsy is done to diagnose childhood non-Hodgkin lymphoma.
Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.

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:

Lymph: Colorless, watery fluid that carries white blood cells called lymphocytesthrough the lymph system. Lymphocytes protect the body against infections and the growth of tumors. There are three types of lymphocytes:
- B lymphocytes that make antibodies to help fight infection.
- T lymphocytes that help B lymphocytes make the antibodies that help fight infection.
- Natural killer cells that attack cancer cells and viruses.
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 are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, 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.

Lymph system; drawing shows the lymph vessels and lymph organs including the lymph nodes, tonsils, thymus, spleen, and bone marrow. One inset shows the inside structure of a lymph node and the attached lymph vessels with arrows showing how the lymph (clear fluid) moves into and out of the lymph node. Another inset shows a close up of bone marrow with blood cells. — 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:

The main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma.

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:

Burkitt and Burkitt-like lymphoma/leukemia: Burkitt lymphoma and Burkitt leukemia are different forms of the same disease. Burkitt lymphoma/leukemia is an aggressive (fast-growing) disorder of B lymphocytes that is most common in children and young adults. It may form in the abdomen, Waldeyer's ring, testicles, bone, bone marrow, skin, or central nervous system (CNS). Burkitt leukemia may start in the lymph nodes as Burkitt lymphoma and then spread to the blood and bone marrow, or it may start in the blood and bone marrow without forming in the lymph nodes first.
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 MYC gene is found.
Diffuse large B-cell lymphoma: Diffuse large B-cell lymphoma is the most common type of non-Hodgkin lymphoma. It is a type of B-cell non-Hodgkin lymphoma that grows quickly in the lymph nodes. The spleen, liver, bone marrow, or other organs are also often affected. Diffuse large B-cell lymphoma occurs more often in adolescentsthan in children.
Primary mediastinal B-cell lymphoma: A type of lymphoma that develops from B cells in the mediastinum (the area behind the breastbone). It may spread to nearby organs including the lungs and the sac around the heart. It may also spread to lymph nodes and distant organs including the kidneys. In children and adolescents, primary mediastinal B-cell lymphoma occurs more often in older adolescents.

Lymphoblastic lymphoma

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 genethat 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:

Pediatric-type follicular lymphoma : In children, follicular lymphoma occurs mainly in males. It is more likely to be found in one area and does not spread to other places in the body. It usually forms in the tonsils and lymph nodes in the neck, but may also form in the testicles, kidney, gastrointestinal tract, and salivary gland.
Marginal zone lymphoma : Marginal zone lymphoma is a type of lymphoma that tends to grow and spread slowly and is usually found at an early stage. It may be found in the lymph nodes or in areas outside the lymph nodes. Marginal zone lymphoma found outside the lymph nodes in children is called mucosa-associated lymphoid tissue (MALT) lymphoma. MALT may be linked to Helicobacter pylori infection of the gastrointestinal tract and Chlamydophila psittaci infection of the conjunctival membranewhich lines the eye.
Primary central nervous system (CNS) lymphoma : Primary CNS lymphoma is extremely rare in children.
Peripheral T-cell lymphoma : Peripheral T-cell lymphoma is an aggressive (fast-growing) non-Hodgkin lymphoma that begins in mature T lymphocytes. The T lymphocytes mature in the thymus gland and travel to other parts of the lymph system, such as the lymph nodes, bone marrow, and spleen.
Cutaneous T-cell lymphoma : Cutaneous T-cell lymphoma begins in the skin and can cause the skin to thicken or form a tumor. It is very rare in children, but is more common in adolescents and young adults. There are different types of cutaneous T-cell lymphoma, such as cutaneous anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, gamma-delta T-cell lymphoma, and mycosis fungoides. Mycosis fungoides rarely occurs in children and adolescents.

Past treatment for cancer and having a weakened immune system affect the risk of having childhood non-Hodgkin lymphoma.

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:

Past treatment for cancer.
Being infected with the Epstein-Barr virus or human immunodeficiency virus (HIV).
Having a weakened immune system after a transplant or from medicines given after a transplant.
Having certain inherited diseases (such as DNA repair defect syndromes which include ataxia-telangiectasia, Nijmegen breakage syndrome, and constitutional mismatch repair deficiency).

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:

Lymphoproliferative disease associated with primary immunodeficiency.
HIV-associated non-Hodgkin lymphoma.
Post-transplant lymphoproliferative disease.

Signs of childhood non-Hodgkin lymphoma include breathing problems and swollen lymph nodes.

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:

Trouble breathing.
Wheezing.
Coughing.
High-pitched breathing sounds.
Swelling of the head, neck, upper body, or arms.
Trouble swallowing.
Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin.
Painless lump or swelling in a testicle.
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.
Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body, including electrolytes, lactate dehydrogenase (LDH), uric acid, blood urea nitrogen (BUN), creatinine, and liver function values. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of cancer.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
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Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
PET scan (positron emission tomography scan): A procedure to find malignanttumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.
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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.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
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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 procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap.
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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.
Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
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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).

A biopsy is done to diagnose childhood non-Hodgkin lymphoma.

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:

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.

The procedure used to remove the sample of tissue depends on where the tumor is in the body:

Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone.
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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.
Mediastinoscopy : A surgical procedure to look at the organs, tissues, and lymph nodes between the lungs for abnormal areas. An incision (cut) is made at the top of the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It also has a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer.
Anterior mediastinotomy : A surgical procedure to look at the organs and tissues between the lungs and between the breastbone and heart for abnormal areas. An incision (cut) is made next to the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It also has a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. This is also called the Chamberlain procedure.
Thoracentesis : The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.

If cancer is found, the following tests may be done to study the cancer cells:

Immunohistochemistry : A laboratory test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.
Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This type of test is used to find certain gene changes.
Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose specific types of lymphoma by comparing the cancer cells to normal cells of the immune system.