Stages of Non-Hodgkin Lymphoma in Children

What is the stage of a lymphoma?

A lymphoma's stage tells you how much and where the cancer is in the body when it is first diagnosed. Along with the type of non-Hodgkin lymphoma (NHL), the stage is important in determining the treatment options and prognosis (outlook) for a child or teen with NHL.

The stage is based on the results of physical exams, biopsies, and imaging tests (CT scan, PET scan, etc.), which are described in Tests for Non-Hodgkin Lymphoma in Children.

A staging system describes how far a cancer has spread in a standard way. The system most often used to describe the spread of NHL in children is the International Pediatric Non-Hodgkin Lymphoma Staging System (IPNHLSS), which is a modified version of the older St. Jude staging system. This system is different from the one used for lymphomas in adults.

International Pediatric Non-Hodgkin Lymphoma Staging System (IPNHLSS)

The IPNHLSS divides childhood NHL into 4 stages:

• Stage I and II lymphomas are usually 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 (belly).

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 in more than one set of lymph nodes, all of which are either above or below the diaphragm (the thin 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. It might or might not have reached nearby lymph nodes.

Stage III

For stage III lymphomas, one of the following applies:

• The lymphoma started in the chest (usually in the thymus, the 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 removed completely by surgery.
• The lymphoma is next to the spine (and may be elsewhere as well).
• The lymphoma is more than one tumor outside of the lymph nodes, which might be above or below the diaphragm (the thin breathing muscle that separates the chest and abdomen). This might include tumors in the bones or skin.
• The lymphoma is in more than one set of lymph nodes above and below the diaphragm. For example, the lymphoma is in both underarm and groin lymph nodes.
• The lymphoma is a single tumor in a bone, and it is in a nearby area as well (either in or outside the lymph nodes).

Stage IV

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

Lymphoma staging can be confusing. If you are unsure about what it means for your child, ask your child’s doctor to explain it to you.

Other prognostic factors

Along with the stage of the lymphoma (and the type of lymphoma), some other factors can also affect a child’s outlook (prognosis). These include:

• The child’s age (younger children tend to do better.)
• The blood LDH level (children with lower LDH levels tend to do better.)
• Where the lymphoma is in the body
• How well the lymphoma responds to initial treatment

If you have questions about what these prognostic factors might mean for your child, ask your child’s doctor to explain them to you.