Childhood Lymphoma

Lymphomas are cancers of the lymphatic system, a key part of body’s immune system. The lymphatic system includes a series of olive-sized nodes that produce lymph, a fluid that carries disease-fighting white blood cells (known as lymphocytes) throughout the body, and the tubes that carry this fluid.

There are two types of lymphocytes affected by lymphoma: T cells and B cells, although B cell lymphomas are much more common.

There are several types of lymphoma, classified by how the cells appear under a microscope. In Hodgkin's lymphoma, the disease is defined by the presence of Reed Sternberg cells, which are large cells that can have more than one nucleus. These cells grow and divide more quickly and live longer than normal cells. They also produce substances that encourage more healthy cells to gather in the lymph nodes. These healthy cells themselves produce substances that encourage the growth of Reed Sternberg cells. There are several subtypes of Hodgkin’s Lymphoma, but the vast majority are classical Hodgkin's lymphoma.

Non-Hodgkin’s lymphoma (NHL), or simply lymphoma, does not have Reed Sternberg cells. There are several subtypes of NHL, including:

• Burkitt's Lymphoma (BL) affects B cell lymphocytes. It is one of the fastest-growing cancers.
• Lymphoblastic Lymphoma (LBL) mostly affects T cell lymphocytes and is similar to acute lymphoblastic leukemia (ALL). It makes up about one-third of all childhood NHL, and is more common in boys.
• Large Cell Lymphoma (LCL) includes two subtypes: diffuse large B-cell lymphoma (DLBCL) mostly affects pre-adolescent and teens; and anaplastic large cell lymphoma (ALCL) is more common in adolescents.

NHL is also classified by how fast it spreads. Nearly all cases of pediatric NHL are the aggressive form of the disease.

Childhood lymphoma risk factors

A risk factor is anything that increases the chances of a person developing a disease. 

There are many different types of lymphoma, so not every risk factor applies to every type.  In addition, most pediatric lymphoma patients don't have any of the risk factors listed here (other than race and gender). Nonetheless, the disease's risk factors include:

• Gender: Hodgkin's lymphoma is more predominant in males than females.
• Race: Whites are more likely to contract the disease than other groups.
• Infections including Epstein-Barr virus, which causes mononucleosis, and HIV
• Immune System diseases, including lupus and rheumatoid arthritis
• Immune deficiency syndromes, including:
  • Ataxia-telangiectasia
  • Bloom syndrome
  • Common variable immunodeficiency
  • Severe combined immunodeficiency syndrome (SCID)
  • Wiskott-Aldrich syndrome
• Taking immunosuppressants due to organ transplant
• Having a parent or sibling with the disease

Some cases of lymphoma can be passed down from one generation to the next. Genetic counseling may be right for you. Learn more about the risk to you and your family on our genetic testing page.

Specific lymphoma symptoms in children vary according to disease type, but most have the following symptoms in common:

• Painless swelling or enlargement of lymph nodes, especially in the neck, armpits and groin
• Excessive night sweats
• Unexplained fever
• Undetermined weight loss
• Loss of appetite
• Itchy skin
• Fatigue

In addition, non-Hodgkin’s lymphoma symptoms can include:

• Swollen abdomen (when the disease started in belly)
• Feeling full after a small meal
• Shortness of breath or cough (when the disease started in the chest)

Most of the time, these symptoms are not caused by leukemia. However, it is important to discuss any symptoms with your doctor, since they may signal other health problems.

A diagnosis for lymphoma often starts with the disease’s symptoms leading to a trip to the doctor. A thorough physical exam then leads to tests that give a definitive diagnosis. Because childhood lymphomas can be difficult to characterize, several tests may have to be done.

Most lymphoma diagnoses start with blood tests, followed by a lymph node biopsy . During this procedure, all or part of a lymph node will be removed and then studied under a microscope. If the lymph node is superficial (close to the surface of the skin), the biopsy can be done with a local anesthetic, meaning that the biopsy area is numbed, but the patient remains awake.

Surgery, in which the patient is put to sleep, may be required if the suspicious lymph node is deeper in the body.

Other tests that can be used to determine the type and stage of the disease are:

• Imaging exams, including CT scans, PET scans and X-ray
• Blood tests that measure the patient’s levels of red blood cells, white blood cells, platelets and hemoglobin
• Blood chemistry studies

Childhood Lymphoma Staging

Following a diagnosis of childhood lymphoma, various 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. 

Stages of Childhood Lymphoma in Children

(Source: National Cancer Institute)

Non-Hodgkin's lymphoma staging

The following stages are used for childhood non-Hodgkin's lymphomas, which include Burkitt's lymphoma, lymphoblastic lymphoma and large-cell lymphomas.

Stage 1: Cancer is found:

• In one group of lymph nodes; or
• In one area outside the lymph nodes.
• No cancer is found in the abdomen or mediastinum (area between the lungs).

Stage 2: Cancer is found:

• In one area outside the lymph nodes and in nearby lymph nodes; or
• In two or more areas above or below the diaphragm, and may or may not have spread to nearby lymph nodes; or
• To have started in the stomach or intestines and can be completely removed by surgery. Cancer may or may not have spread to certain nearby lymph nodes.

Stage 3: Cancer is found:

• In at least one area above the diaphragm and in at least one area below the diaphragm; or
• To have started in the chest; or
• To have started in the abdomen and spread throughout the abdomen, and cannot be completely removed by surgery; or
• In the area around the spine

Stage 4: Cancer is found in the bone marrow, brain or cerebrospinal fluid. Cancer may also be found in other parts of the body.

Treatment for childhood non-Hodgkin's 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. Stage I and stage II are usually considered low-stage.
• High-stage lymphoma has spread beyond the area in which it began. Stage III and stage IV are usually considered high-stage.

Recurrent childhood non-Hodgkin's lymphoma is cancer that has recurred (come back) after it has been treated. It may come back in the lymph system or in other parts of the body.

Hodgkin's lymphoma staging

Stages of childhood Hodgkin's lymphoma may include A, B, E, and S:

• A: The patient has no symptoms.
• B: The patient has symptoms such as fever, weight loss or night sweats.
• E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system.
• S: Cancer is found in the spleen.

Stage 1 is divided into stage I and stage IE:

• Stage I: Cancer is found in one or more lymph nodes in one lymph node group.
• Stage IE: Cancer is found outside the lymph nodes in one organ or area.

Stage 2 is divided into stage II and stage IIE:

• Stage II: Cancer is found in two or more lymph node groups above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).
• Stage IIE: Cancer is found in one or more lymph node groups above or below the diaphragm and outside the lymph nodes in a nearby organ or area.

Stage 3 is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S:

• Stage III: Cancer is found in one or more lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen).
• Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area.
• Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen.
• Stage IIIE+S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen.

Stage 4: the cancer:

• Is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or
• Is found outside the lymph nodes in one organ and has spread to lymph nodes far away from that organ; or
• Is found in the lung, liver or bone marrow

Our approach

At MD Anderson's Children's Cancer Hospital, we know your child's health and well being are your number one concern. Our renowned experts customize your child's lymphoma treatment utilizing the most advanced treatments and techniques with the least impact on your child's growing body.

As part of one of the world's most active cancer centers, Children's Cancer Hospital has remarkable experience and skill in these types of cancer. This can make a difference in your child's outcome.

Customized care

A team of specially trained physicians follows your child throughout treatment, all the way to survivorship. They communicate closely with each other, and with you, to ensure comprehensive, personalized care. They are supported by full complement of health care professionals dedicated to your child's treatment, including nurses, physician assistants, therapists and others.

Children's Cancer Hospital offers clinical trials for innovative new treatments for lymphoma. Behind the scenes we are working on groundbreaking basic science research to change the future of pediatric cancer.

Treating the whole child

Children's Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and family's experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child.

And at Children's Cancer Hospital, your child benefits from the resources and expertise of one of the nation's top cancer centers.

Our childhood lymphoma treatment

The lymph system carries fluids throughout the body, which allows lymphoma to spread easily. Chemotherapy can also travel throughout the body easily. That makes it the most common treatment for lymphoma. Chemotherapy can be administered in four ways:

• Orally (taking pills by mouth)
• Intravenously (injected into the vein)
• Intrathecal (injected directly into the cerebrospinal fluid)
• Subcutaneously (beneath the skin)

Radiation therapy (also called radiotherapy) uses high-energy beams to destroy cancer cells. It is rarely used to threat non-Hodgkin's lymphoma, and used only some of the time to treat Hodgkin's lymphoma.

It is sometimes used in conjunction with chemotherapy to treat large areas, or to minimize discomfort caused by cancerous lymph nodes that are affecting nearby organs or structures. In can also be used to treat tumors that have formed in the central nervous system.

Surgery is also an option for some lymphomas. It can be used when tumors are causing severe and immediate problems, like intestinal blocks, severe pain from compressed nerves and organs, or breathing troubles. In rare cases, it can be used as a form of curative therapy.