What Is Childhood Hodgkin Lymphoma?
Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
Childhood Hodgkin lymphoma is a type of cancer that develops 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 lymphocytes through the lymph system. 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 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 tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. Hodgkin lymphoma often occurs in adolescents 15 to 19 years of age. The treatment for children and adolescents is different than treatment for adults.
What Are the Types of Childhood Hodgkin Lymphoma?
There are two types of childhood Hodgkin lymphoma. The two types of childhood Hodgkin lymphoma are:
- Classical Hodgkin lymphoma
- Nodular lymphocyte-predominant Hodgkin lymphoma
Classical Hodgkin lymphoma is divided into four subtypes, based on how the cancer cells look under a microscope:
- Lymphocyte-rich classical Hodgkin lymphoma
- Nodular sclerosis Hodgkin lymphoma
- Mixed cellularity Hodgkin lymphoma
- Lymphocyte-depleted Hodgkin lymphoma
Epstein-Barr virus infection increases the risk of childhood 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.
Risk factors for childhood Hodgkin lymphoma include the following:
- Being infected with the Epstein-Barr virus
- Being infected with the human immunodeficiency virus (HIV)
- Having certain diseases of the immune system
- Having a personal history of mononucleosis ("mono")
- Having a parent or sibling with a personal history of Hodgkin lymphoma
Being exposed to common infections in early childhood may decrease the risk of Hodgkin lymphoma in children because of the effect it has on the immune system.
What Are the Signs and Symptoms of Childhood Hodgkin Lymphoma?
Signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.
These and other signs and symptoms may be caused by childhood Hodgkin lymphoma or by other conditions.
Check with your child's doctor if your child has any of the following:
- Painless, swollen lymph nodes near the collarbone or in the neck, chest, underarm, or groin
- Fever for no known reason
- Weight loss for no known reason
- Night sweats
- Fatigue
- Anorexia
- Itchy skin
- Pain in the lymph nodes after drinking alcohol
Fever, weight loss, and night sweats are called B symptoms.
How Is Childhood Hodgkin Lymphoma Diagnosed?
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.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, or pelvis, 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.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor 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.
- 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.
- Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells.
- 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.
- 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. The sedimentation rate is a measure of how much inflammation is in the body. A higher than normal sedimentation rate may be a sign of lymphoma. Also called erythrocyte sedimentation rate, sed rate, or ESR.
- Lymph node biopsy: The removal of all or part of a lymph node. The lymph node may be removed during an image-guided CT scan or a thoracoscopy, mediastinoscopy, or laparoscopy. One of the following types of biopsies may be done:
- Excisional biopsy: The removal of an entire lymph node.
- Incisional biopsy: The removal of part of a lymph node.
- Core biopsy: The removal of tissue from a lymph node using a wide needle.
- Fine-needle aspiration (FNA) biopsy: The removal of tissue from a lymph node using a thin needle. A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma.
The following test may be done on tissue that was removed:
- 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 the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system.
What Factors Affect the Treatment Options and Prognosis for Childhood Hodgkin Lymphoma?
- The stage of the cancer
- The size of the tumor
- Whether there are B symptoms at diagnosis
- The type of Hodgkin lymphoma
- Certain features of the cancer cells
- Whether there are too many white blood cells or too few red blood cells at the time of diagnosis
- How well the tumor responds to initial treatment with chemotherapy
- Whether the cancer is newly diagnosed or has recurred (come back)
- The child's age and sex
- The risk of long-term side effects
- Presence of Reed-Sternberg cells (Reed-Sternberg cells are large, abnormal lymphocytes that may contain more than one nucleus and are found in Hodgkin lymphoma)
What Are the Stages of Childhood Hodgkin Lymphoma?
After childhood Hodgkin 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:
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, or pelvis, 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.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor 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.
- 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.
- 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. A pathologist views the bone marrow and bone under a microscope to look for abnormal cells.
There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood:
- Tissue. The cancer spreads from where it began by growing into nearby areas.
- Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
- Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Stages of childhood Hodgkin lymphoma may include A, B, E, and S. Childhood Hodgkin lymphoma may be described as follows:
- A: The patient does not have B symptoms (fever, weight loss, or night sweats).
- B: The patient has B symptoms.
- E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an area of the lymph system affected by the cancer.
- S: Cancer is found in the spleen.
The following stages are used for childhood Hodgkin lymphoma:
Stage I
Stage I is divided into stage I and stage IE.
- Stage I: Cancer is found in one of the following places in the lymph system: One or more lymph nodes in one lymph node group.
- Waldeyer's ring
- Thymus
- Spleen
- Stage IE: Cancer is found outside the lymph system in one organ or area.
Stage II
Stage II is divided into stage II and stage IIE.
- Stage II: Cancer is found in two or more lymph node groups either 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 either above or below the diaphragm and outside the lymph nodes in a nearby organ or area.
- Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area.
Stage III
Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S.
- Stage III: Cancer is found in 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 IV
In stage IV, 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 areas far away from that organ; or
- is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas.
Untreated Hodgkin lymphoma is divided into risk groups. Untreated childhood Hodgkin lymphoma is divided into risk groups based on the stage, size of the tumor, and whether the patient has B symptoms (fever, weight loss, or night sweats). The risk group is used to plan treatment.
- Low-risk childhood Hodgkin lymphoma
- Intermediate-risk childhood Hodgkin lymphoma
- High-risk childhood Hodgkin lymphoma
Primary Refractory/Recurrent Hodgkin Lymphoma in Children and Adolescents
Primary refractory Hodgkin lymphoma is lymphoma that continues to grow or spread during treatment.
Recurrent Hodgkin 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.
What Is the Treatment for Childhood Hodgkin Lymphoma?
There are different types of treatment for children with Hodgkin lymphoma. Different types of treatment are available for children with Hodgkin 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. Some clinical trials are open only to patients who have not started treatment.
Children with Hodgkin lymphoma should have their treatment planned by a team of health care providers who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer.
The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists:
- Pediatrician
- Medical oncologist/hematologist
- Pediatric surgeon
- Radiation oncologist
- Endocrinologist
- Pediatric nurse specialist
- Rehabilitation specialist
- Psychologist
- Social worker
- Child-life specialist
The treatment of Hodgkin lymphoma in adolescents and young adults may be different than the treatment for children. Some adolescents and young adults are treated with an adult treatment regimen.
Children and adolescents may have treatment-related side effects that appear months or years after treatment for Hodgkin lymphoma.
Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Because late effects affect health and development, regular follow-up exams are important.
Late effects of cancer treatment may include:
- Physical problems that affect the following:
- Changes in mood, feelings, thinking, learning, or memory
- Second cancers (new types of cancer)
For female survivors of Hodgkin lymphoma, there is an increased risk of breast cancer. This risk depends on the amount of radiation therapy they received to the breast during treatment and the chemotherapy regimen used. The risk of breast cancer is decreased if these female survivors also received radiation therapy to the ovaries.
It is suggested that female survivors who received radiation therapy to the breast have a mammogram once a year starting 8 years after treatment or at age 25 years, whichever is later. Female survivors of childhood Hodgkin lymphoma who have breast cancer have an increased risk of dying from the disease compared to patients with no history of Hodgkin lymphoma who have breast cancer.
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments.
Treatment options include:
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug.
The way the chemotherapy is given depends on the risk group. For example, children with low-risk Hodgkin lymphoma receive fewer cycles of treatment, fewer anticancer drugs, and lower doses of anticancer drugs than children with high-risk lymphoma.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following:
- Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor.
- Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles.
Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Radiation therapy may be given, based on the child’s risk group and chemotherapy regimen.
External radiation therapy is used to treat childhood Hodgkin lymphoma. The radiation is given only to the lymph nodes or other areas with cancer. Internal radiation therapy is not used to treat Hodgkin lymphoma.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and proteasome inhibitor therapy are being used in the treatment of childhood Hodgkin lymphoma.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
In children, rituximab may be used to treat refractory or recurrent Hodgkin lymphoma. Brentuximab, nivolumab, pembrolizumab, and atezolizumab are monoclonal antibodies being studied to treat children.
Proteasome inhibitor therapy is a type of targeted therapy that blocks the action of proteasomes (proteins that remove other proteins the body no longer needs) in cancer cells and may prevent the growth of tumors.
Bortezomib is a proteasome inhibitor used to treat refractory or recurrent childhood Hodgkin lymphoma.
Surgery
Surgery may be done to remove as much of the tumor as possible for localized nodular lymphocyte-predominant childhood Hodgkin lymphoma.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy 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.
Proton Beam Radiation Therapy
Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to make radiation. This type of radiation therapy may help lessen the damage to healthy tissue near the tumor.
Clinical Trials
Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated.
Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
For patients who receive chemotherapy alone, a PET scan may be done 3 weeks or more after treatment ends. For patients who receive radiation therapy last, a PET scan should not be done until 8 to 12 weeks after treatment.
Childhood Hodgkin Lymphoma Treatment by Risk Group
Low-Risk Classical Childhood Hodgkin Lymphoma
Treatment of low-risk classical childhood Hodgkin lymphoma may include the following:
- Combination chemotherapy
- Radiation therapy may also be given to the areas with cancer
Intermediate-Risk Classical Childhood Hodgkin Lymphoma
Treatment of intermediate-risk classical childhood Hodgkin lymphoma may include the following:
- Combination chemotherapy
- Radiation therapy may also be given to the areas with cancer
High-Risk Classical Childhood Hodgkin Lymphoma
Treatment of high-risk classical childhood Hodgkin lymphoma may include the following:
- Higher dose combination chemotherapy
- Radiation therapy may also be given to the areas with cancer
- A clinical trial of targeted therapy and combination chemotherapy
- Radiation therapy may also be given to the areas with cancer
Nodular Lymphocyte-Predominant Childhood Hodgkin Lymphoma
Treatment of nodular lymphocyte-predominant childhood Hodgkin lymphoma may include the following:
- Surgery, if the tumor can be completely removed
- Chemotherapy with or without low-dose external radiation therapy
Treatment Options for Primary Refractory/Recurrent Hodgkin Lymphoma in Children and Adolescents
Treatment of primary refractory or recurrent childhood Hodgkin lymphoma may include the following:
- Chemotherapy, targeted therapy (rituximab or brentuximab), or both of these therapies
- High-dose chemotherapy with stem cell transplant using the patient's own stem cells
- Low-dose radiation therapy or monoclonal antibody therapy (brentuximab) may be given after the transplant
- High-dose chemotherapy with stem cell transplant using a donor's stem cells
- Monoclonal antibody therapy (brentuximab) in patients whose disease recurred after a stem cell transplant using the patient's own stem cells
- A clinical trial of a monoclonal antibody (nivolumab, pembrolizumab, or atezolizumab)
- A clinical trial that checks a sample of the patient's tumor for certain gene changes (the type of targeted therapy that will be given to the patient depends on the type of gene change)
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Last updated: Sept. 1, 2017