How is non-Hodgkin lymphoma staged in children?

Once non-Hodgkin lymphoma is diagnosed, tests are done to determine the stage (extent of spread) of the disease. A child’s treatment and prognosis (outlook) depend, to a large extent, on the lymphoma's stage.

Staging is based on the results of the physical exam, biopsies, and imaging tests (CT scan, PET scan, etc.), which are described in the section, "How are non-Hodgkin lymphomas diagnosed in children?"

A staging system is a standard way for the cancer care team to sum up how far a cancer has spread. The staging system most often used to describe the spread of non-Hodgkin lymphoma in children is called the St. Jude staging system. This is different from the staging system used for lymphomas in adults (the Ann Arbor staging system).

St. Jude staging system

The St. Jude system divides non-Hodgkin lymphoma in children into 4 stages. In general, stage I and II lymphomas are considered limited-stage disease and are treated the same way. Stage III and IV lymphomas are usually thought of as advanced-stage disease and are also treated alike.

Stage I

The lymphoma is in only one place, either as a single tumor not in lymph nodes or in lymph nodes in one part of the body (the neck, groin, underarm, etc.). The lymphoma is not in the chest or abdomen.

Stage II

Stage II lymphomas are not in the chest, and one of the following applies:

• The lymphoma is a single tumor and is also in nearby lymph nodes in only one part of the body (the neck, groin, underarm, etc.).
• The lymphoma is more than one tumor and/or in more than one set of lymph nodes, all of which are either above or below the diaphragm (the breathing muscle that separates the chest and abdomen). For example, this might mean nodes in the underarm and neck area are affected but not the combination of underarm and groin nodes.
• The lymphoma started in the digestive tract (usually at the end of the small intestine) and can be removed by surgery. Nearby lymph nodes may or may not be involved.

Stage III

For stage III lymphomas, one of the following applies:

• The lymphoma started in the chest (usually in the thymus or lymph nodes in the center of the chest or the lining of the lung).
• The lymphoma started in the abdomen and has spread too widely within the abdomen to be completely removed by surgery.
• The lymphoma is located next to the spine (and may be elsewhere as well).
• The lymphoma is more than one tumor or in more than one set of lymph nodes that are both above and below the diaphragm. For example, the lymphoma may be in both underarm and groin lymph nodes.

Stage IV

The lymphoma is in the central nervous system (brain or spinal cord) or the bone marrow when it is first found. (If more than 25% of the bone marrow is cancer cells, the cancer is classified as acute lymphoblastic leukemia [ALL] instead of lymphoma.).

Survival rates for childhood non-Hodgkin lymphoma

Advances in treatment have increased the overall survival rates for children with non-Hodgkin lymphoma dramatically in recent decades. The 5-year survival rate is used for many types of cancer to refer to the percentage of patients who live at least 5 years after being diagnosed with cancer. With regard to children with non-Hodgkin lymphoma, those who are still alive and free of disease after 5 years are likely to have been cured, as it rare for these cancers to return after this much time.

Survival rates give doctors a standard way to discuss and compare the prognosis (outlook for recovery) for people with cancer. Some parents may want to know the survival statistics for children in similar situations, while others may not find the numbers helpful, or may even not want to know them. If you would rather not read about the survival rates, please skip to the next section, “How is non-Hodgkin lymphoma treated in children?”

Current survival rates are based on children first diagnosed and treated many years ago. Improvements in treatment since then may mean that the outlook is better for recently diagnosed patients.

Survival statistics can sometimes be useful as a general guide, but they may not accurately reflect any one child's prognosis. A number of factors, including the type of lymphoma, the location and size of the tumor(s), and how well the lymphoma responds to treatment, also affect the outlook. Your child's doctor can tell you if the numbers below apply in your child’s situation.

The rates below are based on the results of several studies that may have used different treatment regimens or included slightly different groups of patients, which is why ranges of numbers are given.

Lymphoblastic lymphoma

With intensive treatment, around 90% 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%.

Burkitt and Burkitt-like lymphoma

Treatment of limited stage (stage I and II) Burkitt lymphomas is usually very successful, with a cure rate of over 90%.

The cure rate for children with more advanced (stage III or IV) Burkitt lymphoma ranges from about 80% to 90%.

Large cell lymphomas

The cure rate is over 90% for limited stage (stage I and II) diffuse large B-cell lymphomas and is slightly lower for anaplastic large cell lymphomas.

The cure rate for children with advanced (stage III or IV) diffuse large B-cell lymphoma ranges from about 80% to 90%. For advanced anaplastic large cell lymphoma, the cure rate is about 60% to 75%.