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Learn MoreNon-Hodgkin lymphoma is the group name for all types of lymphomas except Hodgkin lymphoma. Lymphomas are cancers that begin in the body’s lymphatic system, which is part of the body’s immune system.
The lymphatic system moves infection-fighting white blood cells called lymphocytes throughout the body. As a result, lymphoma can start in many places, including the:
As part of the immune system, the lymphatic system moves infection-fighting white blood cells through the body.
Most non-Hodgkin lymphomas of childhood are different from adult lymphomas. Childhood lymphomas are typically more aggressive, but they are highly treatable.
In the United States, about 800 new cases of non-Hodgkin lymphoma in children and teens are diagnosed each year.
Learn more about leukemia and lymphoma causes, treatment, and side effects.
There are several different types of non-Hodgkin lymphoma. Types of childhood non-Hodgkin lymphoma include:
Burkitt lymphoma is an aggressive (fast-growing) disorder of B lymphocytes. It may form in the abdomen, tonsils, testicles, bone, bone marrow, skin, or central nervous system.
There are three main types of Burkitt lymphoma: sporadic, endemic, and immunodeficiency-related.
A different form of the same disease, Burkitt leukemia may start in the lymph nodes as Burkitt lymphoma and then spread to the blood and bone marrow. Or, it may start in the blood and bone marrow without forming in the lymph nodes first.
Burkitt lymphoma
Treatment depends on a number of factors. It could include a combination of chemotherapy drugs or combination chemotherapy with rituximab.
Newly diagnosed
When planning chemotherapy for newly diagnosed disease, the doctor will consider the intensity of dosage and how long to give medicines (duration) based on a variety of risk factors.
These factors include disease stage, site(s) of disease, and level of lactate dehydrogenase (LDH). Examples of commonly used medicines to treat childhood Burkitt lymphoma are:
Some patients may also receive rituximab. The duration ranges from 3 to 8 months depending on risk factors.
Relapsed
Relapsed disease is generally treated with chemotherapy and rituximab followed by bone marrow transplant. A common chemotherapy plan used for relapsed disease is RICE (rituximab, ifosfamide, carboplatin, and etoposide). New targeted therapy strategies are currently under investigation.
Treatment of relapsed Burkitt lymphoma may include a stem cell transplant.
Prognosis depends on a number of factors, including stage, where the disease is located and the serum level of lactate dehydrogenase (LDH). Levels are higher than normal in some patients with lymphoma. The five-year survival rate for limited stage (Stage I and II) childhood Burkitt lymphoma is more than 90 percent.
The five-year survival rate for more advanced (Stage III or IV) childhood Burkitt lymphoma ranges from about 80 to 90 percent.
Diffuse large B-cell lymphoma occurs more often in teens than in children. It is a type of B-cell non-Hodgkin lymphoma that grows quickly in the lymph nodes. It also often affects the spleen, liver, bone marrow, or other organs.
Treatment for childhood diffuse large B-cell lymphoma is generally the same as for Burkitt lymphoma.
Prognosis depends on a number of factors, including the stage of the disease.
The five-year survival rate for limited stage (stage I and II) diffuse large B-cell lymphomas is more than 90 percent.
The five-year survival rate for advanced (stage III or IV) diffuse large B-cell lymphoma ranges from about 80 to 90 percent.
Diffuse large B-cell lymphoma
Primary mediastinal large B-cell lymphoma develops from B cells in the mediastinum. It may spread to nearby organs including the lungs and the sac around the heart. It may also spread to lymph nodes and distant organs including the kidneys. In pediatric cases, primary mediastinal B-cell lymphoma occurs more often in older adolescents.
Treatment options depend on a number of factors. The main treatment for childhood primary mediastinal large B-cell lymphoma is chemotherapy and targeted therapy (rituximab).
The long-term, event-free survival rate for childhood primary mediastinal large B-cell lymphoma is about 70 percent. There is a need for improved treatment approaches which may include novel targeted therapy.
Primary mediastinal large B-cell lymphoma
Lymphoblastic lymphoma mainly affects T-cell lymphocytes. It usually forms in the mediastinum. Symptoms include trouble breathing, wheezing, trouble swallowing, or swelling of the head and neck. It may spread to lymph nodes, bone, bone marrow, skin, the central nervous system, abdominal organs, and other areas. Lymphoblastic lymphoma is a lot like acute lymphoblastic leukemia (ALL).
The medicines and treatment schedule used to treat children with lymphoblastic lymphoma is generally derived from regimens developed to treat acute lymphoblastic leukemia. Agents commonly used include vincristine, prednisone, pegaspargase or Erwinia asparaginase, daunorubicin, methotrexate, mercaptopurine, cytarabine and cyclophosphamide. The length of therapy is about 2 years.
If cancer has spread to the brain and spinal cord, treatment may include radiation and chemotherapy. The pediatric oncologist will review the treatment options including available research protocols. A clinical trial of chemotherapy with different approaches to prevent spread to the central nervous system may be discussed with parents.
Treatment for recurrent lymphoblastic lymphoma may include chemotherapy or high-dose chemotherapy with hematopoietic cell transplant (also called a bone marrow transplant or stem cell transplant).
Treatment of lymphoblastic lymphoma may include a stem cell transplant.
Prognosis depends on a number of factors, including the stage of the disease and the genetic makeup of the cancer.
With intensive treatment, around 90 percent of children with limited stage (stage I or II) lymphoblastic lymphoma can be cured.
The cure rate for more advanced (stage III or IV) lymphoblastic lymphomas is generally higher than 80 percent.
Lymphoblastic lymphoma
Anaplastic large cell lymphoma mainly affects T-cell lymphocytes. It usually forms in the lymph nodes, skin or bone, and sometimes forms in the gastrointestinal tract, lung, tissue that covers the lungs, and muscle.
Treatment options depend on a number of factors, including stage of the disease and the genetic basis of the cancer. Varied approaches have been used to treat children with anaplastic large cell lymphoma. Chemotherapy agents that are commonly used include:
The total duration of therapy ranges from 4 to 12 months.
Treatment options for relapsed anaplastic large cell lymphoma may include chemotherapy, stem cell transplant, a clinical trial of targeted therapy (crizotinib) for children with recurrent anaplastic large cell lymphoma and changes in the ALK gene, a clinical trial with crizotinib with chemotherapy or a clinical trial with an antibody to CD30 linked to another anticancer drug (antibody drug conjugate such as brentuximab), often referred to as immunotherapy.
The 5-year event-free survival for anaplastic large cell lymphoma is about 70 percent.
Anaplastic large cell lymphoma
Signs and symptoms vary depending on where the disease starts in the body:
Doctors may begin to suspect cancer based on the child’s symptoms and from considering the results of a physical examination, medical history and blood tests. Sometimes doctors will order certain diagnostic imaging tests.
These tests may be completed at the local doctor’s office, hospital, or at a cancer center.
Doctors order a biopsy when cancer is suspected. A biopsy is required for diagnosis and will determine exactly what type of lymphoma. During a biopsy, tissue from the suspected tumor is removed and then examined by a pathologist under a microscope.
One of the following types of biopsies may be performed, depending of where the suspected cancer is located:
If lymphoma cells are present, additional tests will be performed on the tissue biopsy to diagnose the specific type of lymphoma. These tests include
If a biopsy confirms cancer, doctors will perform more tests to determine the stage of the disease. The stage indicates whether the cancer has spread to other parts of the body and, if so, how much and how far it has spread.
The care team plans treatment based on the stage of the cancer. It is very important that lymphoma is staged as quickly as possible because these tumors grow rapidly. The higher the stage of the cancer, the more aggressively it needs to be treated.
| Stage | Where Cancer is Found |
|---|---|
| Stage 1 | In one group of lymph nodes In one area outside the lymph nodes No cancer is found in the abdomen or area between the lungs |
| Stage 2 | In one area outside the lymph nodes and in nearby lymph nodes In two or more areas either above or below the diaphragm, and may have spread to nearby lymph nodes To have started in the stomach or intestines and can be completely removed by surgery. Cancer may have spread to certain nearby lymph nodes. |
| Stage 3 | In at least one area above the diaphragm and in at least one area below the diaphragm To have started in the chest To have started in the abdomen and spread throughout the abdomen In the area around the spine |
| Stage 4 | In the bone marrow In the brain Cerebrospinal fluid Other parts of the body. |
The stage of lymphoma indicates whether the cancer has spread to other parts of the body and, if so, how much and how far it has spread.
Chest X-ray with evidence of non-Hodgkin lymphoma in pediatric patient.
Chest X-ray of pediatric non-Hodgkin lymphoma patient shows evidence of disease.
Lateral X-ray of pediatric non-Hodgkin lymphoma with evidence of disease.
Tests that may be performed to help stage childhood non-Hodgkin lymphoma include:
A screening test for HIV (human immunodeficiency virus) is also recommended because lymphomas sometimes develop in patients with this virus. If the test is positive, the patient may require medications to treat HIV.
When determining the best treatment, doctors consider many factors. These include:
The physician and medical team will also consider many factors about the patient, including age and if patients have any existing conditions that might affect their reaction to treatment.
Treatment is based on how well the subtype of lymphoma typically responds to initial therapy. It is called risk-adapted therapy. Risk refers to the risk of cancer not responding successfully to treatment – that it either won’t respond well (refractory) or will return after remission (relapsed).
Patients whose type of lymphoma is typically less responsive to therapies are considered higher than normal risk. These patients are generally given more intense therapies.
Patients with a type of lymphoma that is likely to respond to initial therapy will receive medicines that are likely to have fewer side effects. All patients get medicines and other therapies to help with side effects.
The pediatric oncologist will discuss treatment options with families.
Non-Hodgkin lymphoma tends to be aggressive in children. It is important that treatment begins as soon as possible.
Although significant progress has been made in the treatment of children with lymphoma, 10-30% continue to have refractory or recurrent disease.
But there are treatment options. Many doctors consider intensive chemotherapy with or without treatment with targeted therapy, followed by a hematopoietic cell transplant (also called bone marrow transplant or stem cell transplant).
The subtype of lymphoma is an important factor in making treatment decisions for relapsed disease.
The prognosis (chance of recovery) depends upon
Overall five-year survival rates for childhood non-Hodgkin lymphoma can range from about 70% to more than 90%.
Late effects are side effects that develop after treatment is completed. These include:
Patients should discuss any concerns with their physicians and let them know about any symptoms. Regular, focused follow-up care is extremely important for childhood cancer survivors.
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Reviewed: June 2019