Stages of childhood non-Hodgkin lymphoma (NHL)

Staging describes how much lymphoma there is in the body and where it is when first diagnosed. This is often called the extent of lymphoma. Information from tests is used to find out the size of the tumour, whether the lymphoma has spread from where it first started and where the lymphoma has spread. Your child’s healthcare team uses the stage to plan treatment and estimate the outcome (the prognosis).

The most common staging system for childhood non-Hodgkin lymphoma (NHL) is the St Jude staging system. For childhood NHL there are 4 stages. Often the stages 1 to 4 are written as the Roman numerals I, II, III and IV. Generally, the higher the stage number, the more the lymphoma has spread. Talk to your child’s doctor if you have questions about staging.

The St Jude staging system can be used for all types of childhood NHL, but it is more commonly used to stage lymphoblastic and anaplastic lymphomas.

The lymphoma is in 1 place – either there is 1 tumour outside of the lymph nodes or the lymphoma is in 1 group of lymph nodes (such as the lymph nodes in the neck, groin or underarm). The lymphoma isn’t in the chest or abdomen.

The lymphoma isn’t in the chest, but one of the following applies:

• Lymphoma is in 1 group of lymph nodes and there is a tumour outside of the lymph nodes.
• Lymphoma is in 2 or more groups of lymph nodes that are either all above or all below the diaphragm.
• There are 2 tumours outside of the lymph nodes that are either both above or both below the diaphragm.
• There is a tumour in the digestive tract (the tube that runs from the mouth to the anus) that can be removed by surgery. The lymphoma may have also spread to nearby lymph nodes.

One of the following applies:

• There are 2 tumours outside of the lymph nodes. One tumour is above the diaphragm and the other is below the diaphragm.
• Lymphoma is in 2 or more groups of lymph nodes above and below the diaphragm.
• Lymphoma is in the chest.
• Lymphoma is in the abdomen and can’t be removed by surgery.
• Lymphoma is in an area close to the spine.

Lymphoma is in the brain or spinal cord (called the central nervous system, or CNS), bone marrow or both.

Childhood NHL that starts in B cells are also sorted into risk groups. The risk groups are based on the FAB/LMB system. This helps doctors plan treatment for children with B-cell NHL, such as Burkitt lymphoma, Burkitt-like lymphoma, diffuse large B-cell lymphoma and primary mediastinal B-cell lymphoma.

The lymphoma is stage 1 or 2 and can be removed by surgery.

The lymphoma is in multiple areas outside of the abdomen, or it is stage 1, 2, 3 or 4 and can’t be removed by surgery. If the lymphoma is stage 4, it has spread to the bone marrow and less than 25% of the cells are blasts (lymphoma cells). The lymphoma has not spread to the brain or spinal cord.

The lymphoma is stage 4 and is in the bone marrow, brain or spinal cord. If lymphoma is in the bone marrow, more than 25% of the cells are blasts.

Recurrent, or relapsed, childhood NHL means that the lymphoma has come back after it has been treated. If it comes back in the same place that the lymphoma first started, it’s called local recurrence. If it comes back in tissues or lymph nodes close to where it first started, it’s called regional recurrence. It can also recur in another part of the body. This is called distant metastasis or distant recurrence.

Resistant, or refractory, NHL means that the lymphoma continues to grow or spread during treatment.