Stages of childhood Hodgkin lymphoma

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. The healthcare team will also use the staging information to determine a risk group. Your child’s healthcare team uses the stage and risk group to plan treatment and estimate the outcome (your child’s prognosis).

The most common staging systems for Hodgkin lymphoma (HL) are the Ann Arbor and Lugano staging systems. The systems are similar, and Lugano is based on the Ann Arbor system. In both of these systems 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 the doctor if you have questions about staging.

Doctors stage HL by looking at:

• the number of groups of lymph nodes that have lymphoma
• which lymph nodes have lymphoma and whether they are all above the diaphragm, all below the diaphragm or on both sides of the diaphragm
• whether the lymphoma is found in an organ or tissues other than lymph nodes
• whether the lymphoma has spread in the same area of the body or farther away

HL can also be divided into categories, and the following letters may be added to the stage number:

A – Your child doesn’t have B symptoms (fever, night sweats or weight loss).

B – Your child has B symptoms (unexplained fever that doesn’t go away, drenching night sweats and unexplained weight loss).

E – The lymphoma has grown into nearby tissue outside of the lymph nodes (called an extranodal site).

S – The lymphoma has spread to the spleen.

X – There are large areas of lymphoma (called bulky disease).

Lymphoma is in 1 group of lymph nodes.

Lymphoma is in only 1 area outside of the lymph nodes.

Lymphoma is in 2 or more groups of lymph nodes. The lymph nodes with lymphoma are either all above or all below the diaphragm.

Lymphoma is in lymph nodes either all above or all below the diaphragm. The lymphoma has also grown into tissue near the lymph nodes.

Lymphoma is in lymph nodes both above and below the diaphragm.

Lymphoma is in the spleen and lymph nodes both above and below the diaphragm.

Lymphoma is widespread and found in many areas of the body, such as the lungs, liver, bone or bone marrow. Lymphoma is also usually in the lymph nodes.

Relapsed (recurrent) HL 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 relapse. But it may come back in another part of the body.

Refractory HL means that the lymphoma has not responded to treatment. Refractory HL is also called progressive disease.

When planning treatment for childhood classical HL, the healthcare team will also consider the Children’s Oncology Group (COG) risk groups for childhood HL.

Risk groups are based on the stage of disease, the bulk of the tumour and the presence of B symptoms. B symptoms include fever over 38°C, drenching night sweats and loss of at least 10% of body weight over 6 months.

Treatment is based on these risk groups. Children with lower-risk Hodgkin lymphoma need less treatment than those with higher-risk Hodgkin lymphoma.