Rhabdomyosarcoma - Childhood: Stages and Groups

Approved by the Cancer.Net Editorial Board, 04/2016

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. The tumor’s Clinical Group describes whether it can be removed by surgery. To see other pages, use the menu.

Doctors use both a grouping system and a staging system to describe rhabdomyosarcoma. Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?

  • Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?

IRS Clinical Group 

The Clinical Group is based on whether a surgeon can remove the tumor. The Intergroup Rhabdomyosarcoma Studies (IRS) designed the following grouping and staging guidelines:

Group I: Describes a tumor that can be completely removed by surgery.

Group II: Describes a tumor that has been removed with surgery, but cancer cells remain at the edge of the tissue that surrounded the tumor (called a margin), and/or cancer cells are in the regional lymph nodes (lymph nodes near the site of the tumor).

Group III: Describes a local tumor. A local tumor is a tumor that has not spread outside of the area where it started and cannot be removed by surgery.

Group IV: Describes distant metastases. A distant metastasis is cancer that has spread through the lymph system or blood to other parts of the body.

IRS-modified TNM stage

Stage 1: Describes a local tumor in the orbit (the area near the eye); head and neck area, except for parameningeal sites; or a genitourinary tract tumor, except for a tumor in the bladder or prostate.

Stage 2: Describes a small local tumor in any part of the body not in Stage 1. The tumor is smaller than 5 centimeters (cm), and there is no spread to regional lymph nodes.

Stage 3: Describes a local tumor in any part of the body not included in Stage 1 that is larger than 5 cm in diameter and/or has spread to regional lymph nodes.

Stage 4: Distant metastases are present at diagnosis. The primary tumor can be of any size or location.

Recurrent: Recurrent cancer is cancer that has come back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Source: Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG).    

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu to choose another section to continue reading this guide.