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Once rhabdomyosarcoma (RMS) has been diagnosed and the type of RMS identified, doctors need to assess how much cancer there is and where it has spread. This is known as the stage of the cancer. The stage is one of the most important factors in determining a person's prognosis (outlook). It's also important when deciding on the best treatment options.
Doctors use the results of imaging tests and biopsies and the examination of the organs during surgery to learn how far the cancer has spread. If there is any doubt about the extent of the cancer, more biopsies or other tests may be done.
RMS is staged differently from most other cancers. Doctors first determine 3 key pieces of information:
These factors are used to divide patients into risk groups, which then are used to guide treatment.
The stages and risk groups for RMS can be confusing. If you have any questions about the staging or risk groups, ask the doctor or nurse to explain it to you in a way you understand.
The TNM stage is determined before treatment starts, and is based on 3 key pieces of information:
These factors are combined to determine an overall stage:
The tumor started in a favorable area:
The tumor can be any size. It may have grown into nearby areas and/or spread to nearby lymph nodes, but it has not spread to distant parts of the body.
The tumor started in an unfavorable site:
The tumor is no more than 5 cm (about 2 inches) across and there is no evidence that it has spread to nearby lymph nodes or distant parts of the body.
The tumor started in an unfavorable site:
And one of the following applies:
In either case, the cancer has not spread to distant parts of the body.
The tumor can have started anywhere in the body and can be of any size. It has spread to distant parts of the body such as the lungs, liver, bones, or bone marrow.
The clinical group is based on the extent of the disease and how completely it is removed during initial surgery. The groups are defined as follows.
This group includes children with localized RMS (the cancer has not spread to nearby lymph nodes or to distant sites in the body) that is removed completely by surgery.
This group includes children with localized RMS, but in whom cancer cells have been found at the edges (margins) of the removed specimen (meaning that there may have been a small amount of cancer left behind).
It also includes children with RMS that has spread to the nearby lymph nodes.
These children have tumors that could not be removed completely. Some tumor was left behind that could be seen with the naked eye. The cancer may have spread to nearby lymph nodes, but there is no sign that it has spread to distant organs.
At the time of diagnosis, these children have evidence of distant cancer spread to places such as the lungs, liver, bones, bone marrow, or to distant muscles or lymph nodes.
Using the information about the TNM stage, the clinical group, and the PAX/FOX01 fusion gene status, doctors divide patients into 3 risk groups. This helps doctors decide how aggressive treatment should be.
The risk groups are based on what has been learned from research on people previously treated for RMS. The groups discussed here are based on the most current information, but these may change in the future as safer and more effective treatments are developed.
About 1 in 3 children with RMS falls into the low-risk group. It includes:
About half of children of RMS fall into the intermediate-risk group. It includes:
This group includes:
Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.
National Cancer Institute. Childhood Rhabdomyosarcoma Treatment (PDQ®). 2018. Accessed at www.cancer.gov/types/soft-tissue-sarcoma/hp/rhabdomyosarcoma-treatment-pdq on May 29, 2018.
Okcu MF, Hicks J. Rhabdomyosarcoma in childhood and adolescence: Epidemiology, pathology, and molecular pathogenesis. UpToDate. Accessed at www.uptodate.com/contents/rhabdomyosarcoma-in-childhood-and-adolescence-epidemiology-pathology-and-molecular-pathogenesis on May 29, 2018.
Wexler LH, Skapek SX, Helman LJ. Chapter 31: Rhabdomyosarcoma. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2016.
Last Revised: July 16, 2018
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