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NCI/PDQ^® Patients: Childhood Hodgkin's Lymphoma

National Cancer Institute
Last Modified: January 21, 2004

TABLE OF CONTENTS

• Description
  • What is Hodgkin's lymphoma?

• Stage Explanation
  • Stages of Childhood Hodgkin's lymphoma
    • Stage I
    • Stage II
    • Stage III
    • Stage IV
    • Recurrent

• Treatment Option Overview
  • How childhood Hodgkin's lymphoma is treated
  • Treatment by stage

• Stage I Childhood Hodgkin's Lymphoma

• Stage II Childhood Hodgkin's Lymphoma
  • Stage IIA
  • Stage IIB

• Stage III Childhood Hodgkin's Lymphoma
  • Stage IIIA
  • Stage IIIB

• Stage IV Childhood Hodgkin's Lymphoma

• Recurrent Childhood Hodgkin's Lymphoma

• Changes to This Summary (01/21/2004)

• To Learn More

• About PDQ®

Description

What is Hodgkin's lymphoma?

Hodgkin's lymphoma is a type of lymphoma. Lymphomas are cancers that develop in the lymph system, part of the body's immune system.

The lymph system is made up of thin tubes that branch, like blood vessels, into all parts of the body. Lymph vessels carry lymph, a colorless, watery fluid that contains white blood cells called lymphocytes. Along the network of vessels are groups of small, bean-shaped organs called lymph nodes. Clusters of lymph nodes are found in the underarm, pelvis, neck, chest, and abdomen. The lymph nodes make and store infection-fighting cells. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood), the thymus (a small organ beneath the breastbone), and the tonsils (lymph tissue in the throat) are also part of the lymph system.

Because there is lymph tissue in many parts of the body, Hodgkin's lymphoma can start in almost any part of the body. The cancer can spread to almost any organ or tissue in the body, including the liver, bone marrow (the spongy tissue inside the large bones of the body that makes blood cells), and the spleen.

Lymphomas are divided into 2 general types: Hodgkin's lymphomas and non-Hodgkin's lymphomas. (Refer to the PDQ® summaries on Adult Non-Hodgkin's Lymphoma Treatment and Childhood Non-Hodgkin's Lymphoma Treatment for more information.) This summary covers only childhood Hodgkin's lymphoma. Adult Hodgkin's lymphoma is often treated differently. (Refer to the PDQ® summary on Adult Hodgkin's Lymphoma Treatment for more information.)

Hodgkin's lymphoma is rare in children under 5 years of age. In children under age 10, it is more common in boys than girls. The symptoms of childhood Hodgkin's lymphoma may include any of the following: painless swelling of the lymph nodes in the neck or underarm area that doesn't go away within a few weeks; fever that doesn't go away; night sweats; and weight loss without dieting. If the lymph nodes don't feel normal when examined by a doctor, the doctor may need to cut out a small piece of tissue and look at it under a microscope to see if there are any cancer cells. This is called a biopsy.

Most children with Hodgkin's lymphoma receive combination chemotherapy and low-dose radiation therapy.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in one area or has spread throughout the body), the type of symptoms that are present, and the age and overall condition of the child.

Stage Explanation

Stages of Childhood Hodgkin's lymphoma

Once childhood Hodgkin's lymphoma is found, more tests will be done to find out if the cancer has spread from where it started to other parts of the body. This is called staging. It is important to know the stage of the disease to plan treatment.

The stage of disease may be determined by physical examination, blood tests, and different kinds of x-rays. This is called clinical staging. In some cases, an operation called a laparotomy is performed to determine the stage of the cancer. During this operation, the abdomen is opened to see if the organs inside contain cancer. Small pieces of tissue are removed during the operation and examined under a microscope to see whether they contain cancer. This type of staging is called pathologic staging. Pathologic staging is usually done only when it is needed to help plan treatment. During this operation, the spleen may be removed and the child may receive medicine to prevent infection.

If an operation is not possible, a needle biopsy may be performed. This is a procedure in which tissue is removed with a needle for examination under a microscope.

Each stage of childhood Hodgkin's lymphoma is further divided into A and B categories based on whether the child has symptoms. Children with no symptoms are in the A category, while those who have symptoms (such as fever, weight loss, or night sweats) are in the B category. For example, a child with stage I disease without any symptoms is said to have stage IA disease; a child with stage I disease with symptoms is said to have stage IB disease.

The following stages are used for childhood Hodgkin's lymphoma:

Stage I

Cancer is found in only 1 lymph node area or in only 1 area or organ outside of the lymph nodes.

Stage II

Either of the following means the disease is stage II:

• Cancer is found in 2 or more lymph node areas on the same side of the diaphragm (the thin muscle under the lungs that helps with breathing).
• Cancer is found in only 1 area or organ outside of the lymph nodes and in the lymph nodes around it. Other lymph node areas on the same side of the diaphragm may also have cancer.

Stage III

Cancer is found in lymph node areas on both sides of the diaphragm. The cancer may also have spread to an area or organ near the lymph node areas and/or to the spleen.

Stage IV

Either of the following means the disease is stage IV:

• Cancer has spread in more than 1 spot to an organ or organs outside of the lymph system. Cancer cells may or may not be found in the lymph nodes near these organs.
• Cancer has spread to only 1 organ outside of the lymph system, but lymph nodes far away from that organ are involved.

Recurrent

Recurrent disease means that the cancer has come back after it has been treated. It may come back in the area where it first started or in another part of the body.

Treatment Option Overview

How childhood Hodgkin's lymphoma is treated

There are treatments for all patients with childhood Hodgkin's lymphoma. The most common treatments are radiation therapy and/or chemotherapy, but treatment may be different depending on the stage of the cancer and whether the child has reached full growth. Bone marrow transplants are being studied in clinical trials for certain patients.

Radiation therapy is the use of high-energy x-rays to kill cancer cells and shrink tumors. Radiation for childhood Hodgkin's lymphoma usually comes from a machine outside the body (external beam radiation therapy). Radiation therapy given to the neck, chest, and lymph nodes under the arms is called radiation therapy to the mantle. Radiation therapy given to the mantle and to the lymph nodes in the upper abdomen, the spleen, and the lymph nodes in the pelvis is called total nodal irradiation. Radiation therapy may be used alone or in addition to chemotherapy.

Chemotherapy is the use of drugs to kill cancer cells and shrink tumors. Chemotherapy may be taken by pill, or it may be put into the body by inserting a needle into a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body.

Bone marrow transplantation is a newer type of treatment. Sometimes Hodgkin's lymphoma becomes resistant to treatment with radiation therapy or chemotherapy. Very high doses of chemotherapy may then be used to treat the cancer. Because the high doses of chemotherapy can destroy the bone marrow, marrow is taken from the patient's bones before treatment. The marrow is then frozen and high-dose chemotherapy with or without radiation therapy is given to the patient to treat the cancer. The marrow that was taken from the patient is then thawed and given back through a needle into a vein to replace the marrow that was destroyed. This type of transplant is called an autologous transplant. If the patient is given marrow taken from another person, the transplant is called an allogeneic transplant.

Some patients develop another form of cancer as a result of their treatment for Hodgkin's lymphoma; therefore, regular follow-up evaluations may be made. Female patients who received radiation therapy between the ages of 10 and 16 have an increased risk of breast cancer.

Treatment by stage

Patients may be given vaccination shots against flu, pneumonia, and meningitis both before and every few years after treatment in order to guard against these infections.

Treatment for childhood Hodgkin's lymphoma depends on the type and stage of disease and how the stage was determined, as well as the child's age, symptoms, and general health.

Patients may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or parents may choose to have their child treated in the context of a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. Clinical trials are ongoing in most parts of the country for most stages of childhood Hodgkin's lymphoma. For more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Stage I Childhood Hodgkin's Lymphoma

Treatment depends on whether the disease is stage IA or stage IB and where the cancer is found.

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following

• Chemotherapy with low-dose radiation therapy to areas that contain cancer
• A clinical trial of chemotherapy with or without radiation therapy.
• A clinical trial of chemotherapy alone

If the cancer is above the diaphragm and involves a large part of the chest, treatment may be one of the following

• Chemotherapy followed by radiation therapy to the chest or the mantle
• A clinical trial of chemotherapy plus low-dose radiation therapy

Stage II Childhood Hodgkin's Lymphoma

Treatment depends on whether the disease is stage IIA or stage IIB and where the cancer is found.

Stage IIA

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

• Chemotherapy plus low-dose radiation therapy to areas that contain cancer
• A clinical trial of low-dose chemotherapy with or without radiation therapy
• A clinical trial of chemotherapy alone

If the cancer is above the diaphragm and involves a large part of the chest, treatment may be one of the following:

• Chemotherapy followed by radiation therapy to the chest or the mantle
• A clinical trial of chemotherapy plus low-dose radiation therapy

Stage IIB

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

• Chemotherapy followed by low-dose radiation therapy to areas that contain cancer
• Treatment in a clinical trial

If the cancer is above the diaphragm and involves a large part of the chest, treatment may be one of the following:

• Chemotherapy plus radiation therapy to the chest or the mantle
• A clinical trial of chemotherapy followed by low-dose radiation therapy

Stage III Childhood Hodgkin's Lymphoma

Treatment depends on whether the disease is stage IIIA or stage IIIB.

Stage IIIA

Treatment may be one of the following:

• Chemotherapy alone
• Chemotherapy plus radiation therapy
• A clinical trial of chemotherapy with or without radiation therapy

Stage IIIB

Treatment may be one of the following:

• Chemotherapy alone
• Chemotherapy plus radiation therapy
• Treatment in a clinical trial

Stage IV Childhood Hodgkin's Lymphoma

Treatment may be one of the following:

• Chemotherapy alone
• Chemotherapy plus radiation therapy
• A clinical trial of chemotherapy with or without radiation therapy to the lymph nodes

Recurrent Childhood Hodgkin's Lymphoma

Hodgkin's lymphoma in children and adolescents that does not respond to treatment can be divided into 3 groups: refractory disease (that which does not respond to treatment), disease that recurs within 1 year of complete remission, and disease that recurs more than 1 year after complete remission. Most children with recurrent disease have received previous chemotherapy and different regimens of radiation therapy. Treatment for recurrent/refractory disease may include different chemotherapy regimens, peripheral stem cell transplantation, and/or radiation therapy.

Changes to This Summary (01/21/2004)

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.

Editorial changes were made to this summary.

To Learn More

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About PDQ®

PDQ® is a comprehensive cancer database available on Cancer.gov.

PDQ® is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ® is available online at Cancer.gov, the NCI's Web site. PDQ® is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ® contains cancer information summaries.

The PDQ® database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ® cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ® also contains information on clinical trials.

In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ® and are available online at Cancer.gov. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ® database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on Cancer.gov or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ® database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ® database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.