Lymphoma, Hodgkin's, Childhood: General Information

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

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.

Hodgkin's lymphoma can occur in both children and adults; however, treatment for children may be different than treatment for adults.

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.

Warning Signs

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 health habits and past illnesses and treatments will also be taken.
• Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done:
  • Excisional biopsy: The removal of an entire lymph node.
  • Incisional biopsy or core biopsy: The removal of part of a lymph node.
  • 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, computerized tomography, or computerized axial tomography.
• 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.
• 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 makes 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.

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

• The stage of the cancer.
• The size of the tumor 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 Hodgkin's Lymphoma

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 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.
• Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone 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 IIIE+S.

• 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 that are near or far away from those organs.

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 tumor (tumors 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:
  • Patients with stage I or stage II disease; and
  • No bulky tumors or "b" symptoms.
• Intermediate-risk disease:
  • Patients with stage I or stage II disease, with bulky tumors, or with "b" symptoms; or
  • 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.

Treatment

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.

Children with Hodgkin's lymphoma should have their treatment planned by a team of doctors with expertise 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 doctors who are experts in treating children with Hodgkin's lymphoma and who specialize in certain areas of medicine. These may include the following specialists:

• Medical oncologist/hematologist.
• Pediatric surgeon.
• Radiation oncologist.
• Endocrinologist.
• Pediatric 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 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 spinal column, 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 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 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. 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 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.

Surgery

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

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.

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 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 that a cancer treatment will cause late effects depends on many things, including the following:

• The type of cancer and where it is in the body.
• The child’s age (when treated).
• The type and amount of treatment.
• The area treated.
• Genetic factors or health problems the child had before the cancer.

Regular follow-up by health professionals who are expert in finding and treating late effects is important for the long-term health of childhood cancer survivors. Records about the cancer diagnosis and treatment, including all test results, should be kept by childhood cancer survivors (or their caregivers). This information may be used to help find and treat late effects.

Behaviors that promote health are important for survivors of childhood cancer.

The quality of life enjoyed by cancer survivors may be improved by behaviors that promote their future health and well-being, such as a healthy diet, exercise, and regular medical and dental checkups. These self-care behaviors are especially important for cancer survivors because of their risk of treatment-related health problems. Healthy behaviors may make late effects less severe and lower the risk of other diseases.

Avoiding behaviors that are damaging to health is also important. Smoking, excess alcohol use, and the use of illegal drugs increase the risk of organ damage and, possibly, of second cancers.

Source:  National Cancer Institute