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Childhood Rhabdomyosarcoma Treatment (PDQ®)

Patient Version
Last Modified: 06/20/2014

Stages of Childhood Rhabdomyosarcoma



After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether all the cancer was removed by surgery.

The process used to find out if cancer has spread within the tissue or to other parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic tests to help find out the stage of the disease.

Treatment for childhood rhabdomyosarcoma is based on the stage and the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues, including lymph nodes, removed during surgery, and the edges of the areas where the cancer was removed. This is done to see if all the cancer cells were taken out during the surgery.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if rhabdomyosarcoma spreads to the lung, the cancer cells in the lung are actually rhabdomyosarcoma cells. The disease is metastatic rhabdomyosarcoma, not lung cancer.

Staging of childhood rhabdomyosarcoma is done in three parts.

Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:

The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body:

Stage 1

In stage 1, the tumor is any size, has not spread to lymph nodes, and is found in only one of the following "favorable" sites:

Rhabdomyosarcoma that forms in a "favorable" site has a better prognosis. If the site where cancer occurs is not one of the favorable sites listed above, it is said to be an "unfavorable" site.

Enlarge
Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and lime
Pea, peanut, walnut, and lime show tumor sizes.

Stage 2

In stage 2, cancer is found in an "unfavorable" site (any one area not included in stage 1). The tumor is no larger than 5 centimeters and has not spread to lymph nodes.

Stage 3

In stage 3, cancer is found in an "unfavorable" site (any one area not included in stage 1) and one of the following is true:

  • The tumor is no larger than 5 centimeters and cancer has spread to nearby lymph nodes.
  • The tumor is larger than 5 centimeters and cancer may have spread to nearby lymph nodes.

Stage 4

In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body, such as the lung, bone marrow, or bone.

The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:

Group I

Cancer was found only in the place where it started and it was completely removed by surgery. Tissue was taken from the edges of where the tumor was removed. The tissue was checked under a microscope by a pathologist and no cancer cells were found.

Group II

Group II is divided into groups IIA, IIB, and IIC.

  • IIA: Cancer was removed by surgery but cancer cells were seen when the tissue, taken from the edges of where the tumor was removed, was viewed under a microscope by a pathologist.
  • IIB: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery.
  • IIC: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery. Tissue was taken from the edges of where the tumor was removed. The tissue was checked under a microscope by a pathologist and cancer cells were seen.

Group III

Cancer was partly removed by biopsy or surgery but there is tumor remaining that can be seen with the eye. Cancer has not spread to distant parts of the body.

Group IV

Cancer had spread to distant parts of the body when the cancer was diagnosed.

The risk group is based on the staging system and the grouping system.

The risk group describes the chance that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma. The following risk groups are used:

Low-risk childhood rhabdomyosarcoma

Low-risk childhood rhabdomyosarcoma is one of the following:

  • An embryonal tumor of any size that is found in a "favorable" site. There may be tumor remaining after surgery that can be seen without a microscope. The cancer may have spread to nearby lymph nodes. The following areas are "favorable" sites:
  • An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There may be tumor remaining after surgery that can be seen only with a microscope. The cancer may have spread to nearby lymph nodes.

Intermediate-risk childhood rhabdomyosarcoma

Intermediate-risk childhood rhabdomyosarcoma is one of the following:

  • An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There is tumor remaining after surgery, that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.
  • An alveolar tumor of any size in a "favorable" or "unfavorable" site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.

High-risk childhood rhabdomyosarcoma

High-risk childhood rhabdomyosarcoma may be the embryonal type or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more distant parts of the body.