Key Points for This Section

Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. There are four major types of childhood non-Hodgkin lymphoma. Possible 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. 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.

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.

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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 ¹ for more information.)

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.

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    Bone marrow aspiration and biopsy. After a small area of skin is numbed, a Jamshidi needle (a long, hollow needle) is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.

• 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.
• 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.
• 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.

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.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

An aggressive (fast-growing) type of non-Hodgkin lymphoma that is usually of the T-cell type. The cancer cells express a marker called CD30 or Ki-1 on the surface, and may appear in the lymph nodes, skin, bones, soft tissues, lungs, or liver. Also called ALCL.

A type of immune cell that makes proteins called antibodies, which bind to microorganisms and other foreign substances, and help fight infections. A B cell is a type of white blood cell. Also called B lymphocyte.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

The removal of a small sample of bone marrow (usually from the hip) through a needle for examination under a microscope.

The removal of a sample of tissue from the bone marrow with a needle for examination under a microscope.

A rare, fast-growing type of leukemia (blood cancer) in which too many white blood cells called B lymphocytes form in the blood and bone marrow. It 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 involvement of the lymph nodes. Both Burkitt leukemia and Burkitt lymphoma have been linked to infection with the Epstein-Barr virus.

An aggressive (fast-growing) type of B-cell non-Hodgkin lymphoma that occurs most often in children and young adults. The disease may affect the jaw, central nervous system, bowel, kidneys, ovaries, or other organs. There are three main types of Burkitt lymphoma (sporadic, endemic, and immunodeficiency related). Sporadic Burkitt lymphoma occurs throughout the world, and endemic Burkitt lymphoma occurs in Africa. Immunodeficiency-related Burkitt lymphoma is most often seen in AIDS patients.

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

The removal of a tissue sample with a wide needle for examination under a microscope. Also called core needle biopsy.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

A type of B-cell non-Hodgkin lymphoma (cancer of the immune system) that is usually aggressive (fast-growing). It is the most common type of non-Hodgkin lymphoma, and is marked by rapidly growing tumors in the lymph nodes, spleen, liver, bone marrow, or other organs. Other symptoms include fever, night sweats, and weight loss. There are several subtypes of diffuse large B-cell lymphoma.

A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

The area where the thigh meets the abdomen.

The complex group of organs and cells that defends the body against infections and other diseases.

A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope to check for signs of disease.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

A type of immune cell that is made in the bone marrow and is found in the blood and in lymph tissue. The two main types of lymphocytes are B lymphocytes and T lymphocytes. B lymphocytes make antibodies, and T lymphocytes help kill tumor cells and help control immune responses. A lymphocyte is a type of white blood cell.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

A disease in which cells of the lymphatic system grow excessively. Lymphoproliferative disorders are often treated like cancer.

Any of a large group of cancers of lymphocytes (white blood cells). Non-Hodgkin lymphomas can occur at any age and are often marked by lymph nodes that are larger than normal, fever, and weight loss. There are many different types of non-Hodgkin lymphoma. These types can be divided into aggressive (fast-growing) and indolent (slow-growing) types, and they can be formed from either B-cells or T-cells. B-cell non-Hodgkin lymphomas include Burkitt lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), diffuse large B-cell lymphoma, follicular lymphoma, immunoblastic large cell lymphoma, precursor B-lymphoblastic lymphoma, and mantle cell lymphoma. T-cell non-Hodgkin lymphomas include mycosis fungoides, anaplastic large cell lymphoma, and precursor T-lymphoblastic lymphoma. Lymphomas that occur after bone marrow or stem cell transplantation are usually B-cell non-Hodgkin lymphomas. Prognosis and treatment depend on the stage and type of disease. Also called NHL.

A part of the body that performs a specific function. For example, the heart is an organ.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The lower part of the abdomen, located between the hip bones.

An exam of the body to check for general signs of disease.

A tiny piece of a cell found in the blood that breaks off from a large cell found in the bone marrow. Platelets help wounds heal and prevent bleeding by forming blood clots. Also called thrombocyte.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A cell that carries oxygen to all parts of the body. Also called erythrocyte and RBC.

An organ that is part of the lymphatic system. The spleen makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Removal of fluid from the pleural cavity through a needle inserted between the ribs.

An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.

A group or layer of cells that work together to perform a specific function.

One of two small masses of lymphoid tissue on either side of the throat.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of immune cell. Most white blood cells are made in the bone marrow and are found in the blood and lymph tissue. White blood cells help the body fight infections and other diseases. Granulocytes, monocytes, and lymphocytes are white blood cells. Also called leukocyte and WBC.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.