Staging is a process that tells a doctor if a cancer has spread, and if it has, how far. Staging is an important step in evaluating prognosis and treatment options. Rhabdomyosarcoma is classified into stages, groups, and risk groups. When evaluating treatment options staging enables one institution to compare their treatment program with another hospital. Staging follows a broad criterion that includes tumor size, grade, lymph node spread and distant metastasis. Finally, staging also is used to establish eligibility for some clinical trials. Please refer to the AJCC staging manual for more detail.
The head and neck area, except for parameningeal sites (areas next to the membranes covering the brain, such as the nasal passages and nearby sinuses, middle ear, and the uppermost part of the throat)
A genital or urinary site, except the bladder or prostate gland
Bile ducts (tubes leading from the liver to the intestines)
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.
Stage II
Description
The tumor started in an unfavorable site. Tumor is 5 cm or smaller, has not spread to any lymph nodes, and is found in any site not included in stage one. (bladder, prostate, arm, leg, etc).
Stage III
Description
The tumor started in an unfavorable site. Tumor is 5 cm or smaller, has spread to nearby lymph nodes, and is found in any site not included in stage one.
OR
Tumor is larger than 5 cm, may or may not have spread to nearby lymph nodes, and is found in any site not included in stage one.
Stage IV
Description
The tumor can have started anywhere in the body and can be of any size. Cancer has spread to distant parts of the body, such as the lungs, bones, or bone marrow.
Cancer was found only in the site where it developed and was entirely removed with surgery.
Group IIa
Description
Cancer was completely removed by surgery, but cancer cells were found at the edges of the tissue removed during surgery.
Group IIb
Description
Cancer has spread to nearby lymph nodes. Both the tumor and the malignantlymph nodes were removed by surgery.
Group IIc
Description
Cancer has spread to nearby lymph nodes. Both the tumor and the malignantlymph nodes were removed by surgery, but cancer cells were found at the edges of the tissue removed during surgery.
Group III
Description
Tumor is localized to the primary site with or without spread to lymph nodes, but was not completely removed during surgery (tumor visible to naked eye).
Group IV
Description
Cancer has invaded distant parts of the body at the time of diagnosis.
Low-Risk Group
About one-third of children with rhabdomyosarcoma (RMS) are in the low-risk group. This group includes children whose tumors:
Are TNM stage 1 and in clinical groups I, II, or III, without a PAX/FOXO1 fusion gene
Are stage 2 or 3, in clinical groups I or II, without a PAX/FOXO1 fusion gene.
Intermediate Risk
Description
Stage II or III ERMS; Group III.
Stage I, II, or III ARMS.
Stage IV ERMS less than 10yrs of age.
Intermediate Risk
About half of children of RMS fall into the intermediate-risk group. It includes:
Children with stage 2 or 3 RMS, in clinical group III, whose cancer cells do not have a PAX/FOXO1 fusion gene
Children with stage 1, 2, or 3 RMS (no distant spread), whose cancer cells do have a PAX/FOXO1 fusion gene
Children younger than 10 years of age with widespread (stage 4) RMS, in which the cancer cells do not have a PAX/FOX01 fusion gene.
High Risk
Description
Stage IV (widespread) ARMS
Stage IV ERMS in children more than 10yrs of age.
Children 10 years or older with widespread (stage 4) RMS, whose cancer cells do not have a PAX/FOXO1 fusion gene
Children of any age with widespread (stage 4) RMS, whose cancer cells do have a PAX/FOXO1 fusion gene.
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