Childhood Hodgkin's Lymphoma

Key Points for This Section

  • Childhood Hodgkin's lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
  • There are two types of childhood Hodgkin’s lymphoma.
  • Age, gender, and Epstein-Barr virus infection can affect the risk of developing childhood Hodgkin's lymphoma.
  • Possible signs of childhood Hodgkin's lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.
  • Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin's lymphoma.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

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

Childhood Hodgkin's lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The lymph system 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 tumours.
  • 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 substances in lymph and 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 underarm, pelvis, neck, abdomen, and groin.
  • Spleen: An organ that produces 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.
  • 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 produce lymphocytes.
  • Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow produces white blood cells, red blood cells, and platelets.

Because lymph tissue is found throughout the body, Hodgkin's lymphoma can start in almost any part of the body and spread to almost any tissue or organ in the body.

Lymphomas are divided into two general types: Hodgkin's lymphoma and non-Hodgkin's lymphoma. (Refer to the summary on Childhood Non-Hodgkin's Lymphoma Treatment for more information.)

Hodgkin's lymphoma can occur in both children and adults; however, treatment for children may be different than treatment for adults. (Refer to the summary on Adult Hodgkin's Lymphoma Treatment for more information.)

There are two types of childhood Hodgkin’s lymphoma.

The two types of childhood Hodgkin’s lymphoma are:

  • Classical Hodgkin's lymphoma.
  • Nodular lymphocyte predominant Hodgkin's lymphoma.

Classical Hodgkin's lymphoma is divided into four subtypes, based on how the cancer cells look under a microscope:

  • Lymphocyte-rich classical Hodgkin’s lymphoma.
  • Nodular sclerosis Hodgkin’s lymphoma.
  • Mixed cellularity Hodgkin’s lymphoma.
  • Lymphocyte-depleted Hodgkin’s lymphoma.

Age, gender, and Epstein-Barr virus infection can affect the risk of developing childhood Hodgkin's lymphoma.

Risk factors include the following:

  • Being between the ages of 5 and 14. In children younger than 14 years, it is more common in boys than in girls.
  • Being infected with the Epstein-Barr virus.
  • Having a brother or sister with Hodgkin's lymphoma.

Possible signs of childhood Hodgkin's lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.

These and other symptoms may be caused by childhood Hodgkin's lymphoma or by other conditions. A doctor should be consulted if any of the following problems occur:

  • Painless, swollen lymph nodes in the neck, chest, underarm, or groin.
  • Fever.
  • Night sweats.
  • Weight loss for no known reason.
  • Itchy skin.

Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin's 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 past illnesses and treatments will also be taken.
  • Lymph node biopsy: The removal of all or part of a lymph node that is then viewed under a microscope and checked for signs of disease. One of the following types of biopsies may be done:
    • o Excisional biopsy: The removal of an entire lymph node.
    o Incisional biopsy or core biopsy: The removal of part of a lymph node.
    o Needle biopsy or fine-needle aspiration: The removal of a sample of tissue from a lymph node with a needle.
  • 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, computerised tomography, or computerised axial tomography.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
    • o The number of red blood cells, white blood cells, and platelets.
    o The amount of haemoglobin (the protein that carries oxygen) in the red blood cells.
    o The portion of the blood sample made up of red blood cells.
  • Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
  • 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. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it.
  • Gallium scan: A procedure to detect areas of the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactive material, gallium, is injected into a vein and travels through the bloodstream. The gallium collects in the bones or other tissues (organs) and is detected by a scanner.
  • Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
  • Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out the type of malignant (cancerous) lymphocytes that are causing the lymphoma.

Certain factors affect prognosis (chance of recovery) and treatment options.

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

  • The stage of the cancer.
  • The size of the tumour and how quickly it shrinks after initial treatment.
  • The patient's symptoms when diagnosed.
  • Certain features of the cancer cells.
  • Whether the cancer is newly diagnosed, does not respond to initial treatment, or has recurred (come back).

The treatment options also depend on:

  • The child's age and gender.
  • The risk of long-term side effects.

Most children and adolescents with newly diagnosed Hodgkin's lymphoma can be cured.

Stages of Childhood Hodgkin's Lymphoma

Key Points for This Section

  • After childhood Hodgkin's 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 letters "E" and "S" may be used to describe the stages of childhood Hodgkin's lymphoma.
  • The following stages are used for childhood Hodgkin's lymphoma:
    • o Stage I
    o Stage II
    o Stage III
    o Stage IV
  • Untreated, classical Hodgkin's lymphoma is divided into risk groups.
  • Childhood Hodgkin's lymphoma is staged again after initial chemotherapy.

After childhood Hodgkin's 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. Treatment is based on the stage and other factors that affect prognosis. The following tests and procedures may be used in the staging process:

  • 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). An MRI of the abdomen and pelvis may be done.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumour cells in the body. A small amount of radionuclide 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 tumour cells show up brighter in the picture because they are more active and take up more glucose than normal cells.
  • Bone marrow aspiration and biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for abnormal cells.

The letters "E" and "S" may be used to describe the stages of childhood Hodgkin's lymphoma.

  • 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.

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

Stage I

Stage I is divided into stage I and stage IE.

  • Stage I: Cancer is found in one group of lymph nodes.
  • Stage IE: Cancer is found in one group of lymph nodes and has spread to a nearby organ or tissue that is not part of the lymph system.

Stage II

Stage II is divided into stage II and stage IIE.

  • Stage II: Cancer is found in two or more lymph node groups on the same side of the diaphragm.
  • Stage IIE: Cancer is found in two or more lymph node groups on the same side of the diaphragm and has spread from one of those lymph nodes to a nearby organ or tissue that is not part of the lymph system.

Stage III

Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIS+E.

  • Stage III: Cancer is found in lymph node groups on both sides of the diaphragm.
  • Stage IIIE: Cancer is found in lymph node groups on both sides of the diaphragm and has spread from one of these lymph nodes to a nearby organ or tissue that is not part of the lymph system.
  • Stage IIIS: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen.
  • Stage IIIE+S: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen, and has spread from one of these lymph node groups to a nearby organ or tissue that is not part of the lymph system.

Stage IV

In stage IV:

  • cancer is found throughout one or more organs that are not part of the lymph system and may be in lymph nodes near those organs; or
  • cancer is found throughout one organ that is not part of the lymph system and in lymph nodes far away from that organ.

Untreated, classical Hodgkin's lymphoma is divided into risk groups.

Untreated, classical childhood Hodgkin's lymphoma is divided into risk groups based on the bulk of the tumour (tumours that are 5 centimeters or larger are considered "bulky") and whether the patient has "b" symptoms (fever, weight loss, or night sweats). Treatment is based on the risk group.

  • Low-risk disease:
    • o Patients with stage I or stage II disease; and
    o No bulky tumours or "b" symptoms.
  • Intermediate-risk disease:
    • o Patients with stage I or stage II disease, with bulky tumours, or with "b" symptoms; or
    o Patients with stage III or stage IV disease without "b" symptoms.
  • High-risk disease: Patients with stage III or stage IV disease with "b" symptoms.

Childhood Hodgkin's lymphoma is staged again after initial chemotherapy.

A PET or gallium scan is done after chemotherapy ends to find out how well the chemotherapy worked.

Primary Progressive/Recurrent Hodgkin's Lymphoma in Children and Adolescents

Primary progressive Hodgkin's lymphoma is lymphoma that continues to grow or spread during treatment. Recurrent Hodgkin's lymphoma is cancer that has recurred (come back) after it has been treated. The lymphoma may come back in the lymph system or in other parts of the body, such as the lungs, liver, bones, or bone marrow.

Treatment Option Overview

Key Points for This Section

  • There are different types of treatment for children with Hodgkin's lymphoma.
  • Children with Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.
  • Two types of standard treatment are used:
    • o Chemotherapy
    o Radiation therapy
  • Other types of treatment are being tested in clinical trials. These include the following:
    • o High-dose chemotherapy with stem cell transplant
    o Surgery

    There are different types of treatment for children with Hodgkin's lymphoma.

    Different types of treatment are available for children with Hodgkin's lymphoma. Some treatments are standard 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 Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise in treating childhood cancer.

    Your child's treatment will be overseen by a paediatric oncologist, a doctor who specializes in treating children with cancer. The paediatric oncologist may refer you to other paediatric doctors who have experience and expertise in treating children with Hodgkin's lymphoma and who specialize in certain areas of medicine. These may include the following specialists:

    • Medical oncologist/haematologist.
    • Paediatric surgeon.
    • Radiation oncologist.
    • Endocrinologist.
    • Paediatric nurse specialist.
    • Rehabilitation specialist.
    • Psychologist.
    • Social worker.

    Two 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 the cells 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, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas. Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

    Radiation therapy

    Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. 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. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

    Other types of treatment are being tested in clinical trials. These include the following:

    High-dose chemotherapy with stem cell transplant

    High-dose chemotherapy with stem cell transplant is a method of replacing blood-forming cells destroyed by high doses of anticancer drugs or radiation therapy. 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 therapy 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.

    Surgery

    Surgery may be done to remove as much of the tumour as possible.

    Treatment Options for Children and Adolescents with Hodgkin's Lymphoma

    Low-Risk Childhood Hodgkin's Lymphoma

    Treatment of low-risk childhood Hodgkin's lymphoma may include the following:

    • Combination chemotherapy with low-dose radiation therapy to involved areas.
    • A clinical trial of combination chemotherapy with or without low-dose radiation therapy to involved areas.

    Intermediate-Risk Childhood Hodgkin's Lymphoma

    Treatment of intermediate-risk childhood Hodgkin's lymphoma may include the following:

    • Combination chemotherapy with low-dose radiation therapy to involved areas.
    • A clinical trial of combination chemotherapy with or without low-dose radiation therapy to involved areas.
    • A clinical trial of new combinations of chemotherapy before low-dose radiation therapy to involved areas.

    High-Risk Childhood Hodgkin's Lymphoma

    Treatment of high-risk childhood Hodgkin's lymphoma may include intensive or high-dose combination chemotherapy with low-dose radiation therapy to involved areas.
    Nodular Lymphocyte Predominant Childhood Hodgkin's Lymphoma

    Treatment of nodular lymphocyte predominant childhood Hodgkin's lymphoma may include the following:

    • Combination chemotherapy with low-dose radiation therapy to involved areas.
    • A clinical trial of surgery only, when the lymphoma is stage I and no cancer remains after the surgery.
    • A clinical trial of combination chemotherapy with or without low-dose radiation therapy to involved areas for patients with stage I or stage II.

    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 Primary Progressive/Recurrent Hodgkin's Lymphoma in Children and Adolescents

    Treatment of primary progressive or recurrent childhood Hodgkin's lymphoma may include the following:

    • Chemotherapy with low-dose radiation therapy to involved areas for stage I or stage II non-bulky disease. Adolescent patients who have reached full growth may be treated with standard-dose radiation therapy.
    • High-dose chemotherapy with stem cell transplant with or without radiation therapy.

    Late Effects from Childhood and Adolescent Hodgkin's Lymphoma Treatment

    Children and adolescents may have treatment-related side effects that appear months or years after treatment for Hodgkin's lymphoma. Because of these late effects on health and development, regular follow-up exams are important. Late effects may include problems with the following:

    • Development of sex organs in males.
    • Fertility (ability to have children).
    • Thyroid, heart, or lungs.
    • An increased risk of developing a second primary cancer.
    • Bone growth and development.

    The risk of these long-term side effects will be considered when treatment decisions are made.


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