Rhabdomyosarcoma

After diagnosis, your care team may assign a stage to the cancer. For children, the care team also may assign a clinical group and risk group. Together, these groupings help your care team plan treatment and understand your prognosis.

Staging terms

Stage describes how much cancer there is and how far it has spread. For rhabdomyosarcoma, stages range from 1 to 4. A lower stage usually means the cancer is more limited. Stage 4 means the cancer has spread far from where it started. Where the tumor started also affects the stage. Some areas in the body, such as the head and neck and the area around the eye, have a better outlook.

Healthcare professionals use a TNM system to help assign a stage to the rhabdomyosarcoma. TNM stands for:

• T: The size of the initial tumor and whether it has spread from where it started.
• N: Whether the cancer has spread to nearby lymph nodes.
• M: Whether the cancer has spread to distant parts of the body.

Clinical groups

Rhabdomyosarcoma that is not the pleomorphic type also is assigned to a clinical group. While stage describes the cancer before treatment, a clinical group describes what cancer is left after the first surgery or biopsy. Clinical groups also include the extent of the cancer. Cancer cannot always be fully removed.

• Group 1. The tumor was fully removed. And the cancer has not spread beyond the area in which is started.
• Group 2. The tumor was removed. However, microscopic cancer cells are at the edges of the removed tissue or in nearby lymph nodes. The cancer has not spread beyond nearby lymph nodes.
• Group 3. Some of the tumor that's large enough to be seen with the eye remains in the body after biopsy or surgery. The cancer does not seem to have spread beyond nearby lymph nodes.
• Group 4. Like stage 4, group 4 means the cancer has spread to distant parts of the body.

Risk groups

After staging, for people under age 21, the care team also assigns rhabdomyosarcoma to a risk group. This reflects the risk of rhabdomyosarcoma returning, also called recurring, after treatment.

When assigning a risk group, the care team thinks about:

• Where the tumor started.
• How large the tumor is.
• Whether the cancer has spread, including if nearby lymph nodes have cancer.
• How much tumor is left after surgery or biopsy.
• Whether gene changes are found, especially a FOXO1 fusion.
• A person's age.

The main risk groups are:

• Very low risk and low risk. These cancers have the best outlook and are more likely to be cured.
• Intermediate risk. These cancers often can be cured. But they also have a higher chance of recurrence than cancers that are low or very low risk.
• High risk. These cancers have the highest risk of recurring and a less positive outlook. They are harder to cure.

Your care team can explain what your rhabdomyosarcoma's stage, clinical group and risk group are and what they mean for you and your treatment.

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