Stages of rhabdomyosarcoma

Staging describes or classifies a cancer based on how much cancer there is in the body and where it is when first diagnosed. This is often called the extent of cancer. Information from tests is used to find out where the tumour started, the size of the tumour, which parts of the organ have cancer, whether the cancer has spread from where it first started and where the cancer has spread.

Your healthcare team uses the stage to plan treatment and estimate the outcome (your prognosis). The stage also depends on where the tumour started and whether it’s favourable or unfavourable. The healthcare team also look at other factors like the clinical group and the risk group.

When describing the stage, doctors may use the words localized or metastatic. Localized means that the cancer is only in the area where it started or close to it, including in nearby lymph nodes. Metastatic means in a part of the body farther from the area where the cancer started.

A common staging system for rhabdomyosarcoma is the TNM system. For rhabdomyosarcoma there are 4 stages. Often the stages 1 to 4 are written as the Roman numerals I, II, III and IV. Generally, the higher the stage number, the more the cancer has spread. Talk to your doctor if you have questions about staging.

Find out more about staging cancer.

Where the tumour started

Where the tumour started is also called the site of the primary tumour. Doctors describe it as favourable or unfavourable. Favourable sites have a better (more favourable) prognosis. Unfavourable sites have a poorer (less favourable) prognosis.

Favourable sites include the:

  • area around the eye (called the orbit)
  • eyelid
  • head and neck areas but not areas near the meninges (also called non-parameningeal sites)
  • ovaries, fallopian tubes, uterus, cervix, vagina, area next to the testicles (paratesticular), penis, ureters and urethra

Unfavourable sites include all other sites, such as the:

  • bladder
  • prostate
  • muscles in an arm or leg
  • area around the skull (cranium)
  • head and neck areas close to the meninges (parameningeal), including the nasal passages, paranasal sinuses, middle ear and back of the upper throat
  • muscles in the central part of the body (trunk)
  • back of the abdomen (retroperitoneum)

Stage 1

The cancer is in a favourable site. It has not spread to a part of a body farther from where it started.

Stage 2

The cancer is in an unfavourable site. The tumour is 5 cm or smaller. It may have grown into surrounding areas.

Stage 3

The cancer is in an unfavourable site. The tumour is 5 cm or smaller. It may have grown into surrounding areas. The cancer has spread to nearby lymph nodes.

Or the cancer is in an unfavourable site. The tumour is larger than 5 cm. It may have grown into surrounding areas. The cancer may have spread to nearby lymph nodes.

Stage 4

The cancer is in any site. It has spread to other parts of the body (called distant metastasis). This is also called metastatic cancer.

Recurrent rhabdomyosarcoma

Recurrent rhabdomyosarcoma means that the cancer has come back after it has been treated. If it comes back in the same place that the cancer first started, it’s called local recurrence. If it comes back in tissues or lymph nodes close to where it first started, it’s called regional recurrence. It can also recur in another part of the body. This is called distant metastasis or distant recurrence.

Clinical groups

Clinical groups are assigned after surgery based on whether the cancer has spread and how much of the cancer was removed during surgery. The groups are assigned according to the Intergroup Rhabdomyosarcoma Study group system. Each group is given a number from 1 to 4. Generally, the higher the number, the more the cancer has spread.

Group 1

The tumour is localized. It was completely removed with surgery.

Group 2

The tumour is localized. It was removed with surgery, but cancer cells were found at the margin between the cancer and the surrounding healthy local tissue that was removed with the tumour, nearby lymph nodes or both.

Group 3

The tumour wasn’t completely removed with surgery. The cancer may have spread to nearby lymph nodes but not distant organs.

Group 4

The cancer has spread to other parts of the body, such as the lungs, the liver, the bones, the bone marrow, distant muscles or distant lymph nodes.

Risk groups

Risk groups help the doctor estimate the outcome and plan the best treatment for children diagnosed with rhabdomyosarcoma. These risk groups are assigned by the Soft Tissue Sarcoma Committee of the Children’s Oncology Group.

Risk group

Type of rhabdomyosarcoma


Clinical group




1, 2 or 3

2 or 3 1 or 2



2 or 3



1, 2 or 3

1, 2 or 3


embryonal or alveolar



Expert review and references

  • American Cancer Society. Rhabdomyosarcoma. 2014:
  • Brierley JD, Gospodarowicz MK, Wittekind C (eds.). TNM Classification of Malignant Tumours. 8th ed. Wiley Blackwell; 2017.
  • Imbach P . Soft tissue sarcomas. Imbach P, Kuhne T, Arceci RJ (eds.). Pediatric Oncology: A Comprehensive Guide. 3rd ed. Cham, CH: Springer; 2014: 12:143-160.
  • National Cancer Institute. Childhood RhabdomyosarcomaTreatment (PDQ®) Patient Version. 2014:
  • Wexler LH, Meyer WH, & Helman LJ . Rhabdomyosarcoma. Pizzo, P. A. & Poplack, D. G. (Eds.). Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia: Lippincott Williams & Wilkins; 2011: 31: pp. 923-953.
  • Zinger Kotsubo C . Rhabdomyosarcoma. Baggott C, Fochtman D, Foley GV, Patterson K (eds.). Nursing Care of Children and Adolescents with Cancer and Blood Disorders. 4th ed. APHON; 2011: 31:1054-1070.