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Stages of Childhood Rhabdomyosarcoma
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After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether cancer remains after surgery to remove the tumor.
The process used to find out if cancer has spread within the muscle 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 determine 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.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Staging of childhood rhabdomyosarcoma is done in three related steps.
Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:
- A grouping system.
- A staging system.
- A risk group.
The grouping system is based on whether the cancer has spread and how much cancer remains after surgery to remove the tumor:
Group I
Cancer was only in the place where it started and it was completely removed by surgery. No cancer cells are seen when tissue taken from the edges of where the tumor was removed is viewed under a microscope by a pathologist.
Group II
Group II is divided into groups IIA, IIB, and IIC.
- IIA: Cancer was removed by surgery but there are cancer cells remaining that can be seen only with a microscope.
- 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. There are cancer cells remaining that can be seen only with a microscope.
Group III
Cancer was removed by surgery and there are cancer cells remaining that can be seen without a microscope. Cancer did not spread to distant parts of the body.
Group IV
Cancer had spread to distant parts of the body at the time of diagnosis.
The staging system is based on tumor location and size, and whether it has spread to lymph nodes or distant parts of the body:
Stage 1
In stage 1, cancer is any size, has not spread to lymph nodes, and is found in only one of the following "favorable" sites:
Rhabdomyosarcoma that occurs 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 considered an "unfavorable" site.
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| Pea, peanut, walnut, and lime show tumor sizes. |
Stage 2
In stage 2, cancer is found in any one area not included in stage 1. The tumor is 5 centimeters or smaller and has not spread to lymph nodes.
Stage 3
In stage 3, cancer is found in any one area not included in stage 1 and one of the following is true:
- The tumor is 5 centimeters or smaller 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 risk group is based on both the grouping system and the staging system and is used to plan treatment.
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 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 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, alveolar type, or anaplastic type. It may have spread to nearby lymph nodes and has spread to distant parts of the body.
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