Doctors stage Ewing sarcoma by considering its size, whether it affects lymph nodes, and whether it has spread to other areas. It always has a high grade, meaning that the cancer looks highly atypical.

Ewing sarcoma is a cancer that can develop in the bones and soft tissues around the bones.

This article details how doctors stage Ewing sarcoma using the American Joint Committee on Cancer (AJCC) staging system. It then discusses the difference between localized and metastatic Ewing tumors and staging for bone cancers and soft tissue sarcomas.

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According to the American Cancer Society (ACS), doctors stage Ewing sarcoma with the AJCC staging system.

The AJCC takes into account four main criteria:

  • Tumor size (T): The size of a primary tumor.
  • Lymph nodes (N): The extent to which cancer has spread to nearby lymph nodes.
  • Metastasis (M): Whether the cancer has metastasized (spread) to other organs or body parts.
  • Grade (G): How atypical the cancer cells look (cancer grade from G1 to G3).

Ewing sarcoma is always a high grade cancer. This corresponds to the grade G3.

Learn more about the stages of cancer.

Localized Ewing tumors remain in the area of the body they originally started. Doctors may also consider them localized if they have only spread to nearby tissue, such as the muscles or tendons.

Metastatic Ewing tumors have spread to other parts of the body. Research shows that upon diagnosis, around 1 in 5 people with Ewing sarcoma have metastatic Ewing tumors.

Most commonly, Ewing sarcoma spreads to:

  • other bones
  • bone marrow
  • the lungs

Sometimes, Ewing sarcoma can spread to the liver and lymph nodes.

According to the ACS, the AJCC stages bone cancers in the following way:

AJCC stageStage groupingDescription
1AT1
N0
M0
G1 or GX
• The main tumor is 8 centimeters (cm) or less across (T1).
• The cancer has not spread to nearby lymph nodes (N0) or to distant parts of the body (M0).
• The cancer is low grade (G1), or doctors cannot determine the grade (GX).
1BT2
N0
M0
G1 or GX
Like 1A, except the main tumor is greater than 8 centimeters across (T2).
1B (also)T3
N0
M0
G1 or GX
Like 1A, except there is more than one tumor in the same bone (T3).
2A T1
N0
M0
G2 or G3
Like 1A, except the cancer is high grade (G2 or G3).
2B T2
N0
M0
G2 or G3
• The tumor size is greater than 8 centimeters across (T2).
• The cancer has not spread to nearby lymph nodes (N0) or to distant parts of the body (M0).
• The grade is G2 or G3.
3T3
N0
M0
G2 or G3
• There is more than one tumor in the same bone (T3).
• The cancer has not spread to nearby lymph nodes (N0) or to distant parts of the body (M0).
• The grade is G2 or G3.
4AAny T
N0
M1a
Any G
• The main tumor may be of any size.
• There could be several tumors in one bone (Any T).
• The cancer has not spread to nearby lymph nodes (N0) but has spread to the lungs (M1a).
• The cancer may be of any grade (Any G).
4BAny T
N1
Any M
Any G
• The size is Any T.
• The cancer has spread to nearby lymph nodes (N1) and may have spread to distant organs (Any M).
• The grade is Any G.
4B (also)Any T
Any N
M1b
Any G
• The size is Any T.
• The cancer might have spread to nearby lymph nodes (Any N) and has spread to distant body parts (M1b).
• The grade is Any G.

Learn about the types of bone cancer.

The ACS also details the AJCC staging system for soft tissue sarcomas. Doctors use the following system for sarcomas affecting the trunk, legs, and arms:

AJCC stageStage groupingDescription
1AT1
N0
M0
G1 or GX
• The cancer is no greater than 5 cm (T1).
• It has not spread to nearby lymph nodes (N0) or to distant sites (M0).
• The grade is G1, or doctors cannot determine the grade (GX).
1BT2, T3, or T4
N0
M0
G1 or GX
The cancer has one of these sizes:

• larger than 5 cm, but not larger than 10 cm (T2)
• larger than 10 cm, but not larger than 15 cm (T3)
• larger than 15 cm (T4)

The cancer has not spread to nearby lymph nodes (N0) or to distant parts of the body (M0) and the grade is G1 or GX.
2T1
N0
M0
G2 or G3
• The size is no larger than 5 cm (T1).
• The cancer has not spread to nearby lymph nodes (N0) or to distant parts of the body (M0).
• The grade is G2 or G3.
3A T2
N0
M0
G2 or G3
Like stage 2, except the size is larger than 5 cm but no bigger than 10 cm (T2).
3BT3 or T4
N0
M0
G2 or G3
Like stage 2, except the size is larger than 10 cm but no bigger than 15 cm (T3), or it is larger than 15 cm (T4).
4Any T
N1
M0
Any G
• The size is Any T.
• The cancer has spread to nearby lymph nodes (N1).
• The cancer has not spread to distant sites (M0).
• The grade is Any G.
4 (also)Any T
Any N
M1
Any G
• The size is Any T.
• The cancer has spread to nearby lymph nodes (N1).
• The cancer has spread to distant sites (M0).
• The grade is Any G.

Soft tissue sarcomas can also develop in the retroperitoneum, an area in the back of the abdomen. Doctors stage the cancers in the following ways:

AJCC stageStage groupingDescription
1AT1
N0
M0
G1 or GX
• The size is no larger than 5 cm (T1).
• It has not spread to nearby lymph nodes (N0) or to distant sites (M0).
• The grade is G1 or GX.
1BT2, T3, or T4
N0
M0
G1 or GX
Like 1A, except the size is one of the following:

• larger than 5 cm but not larger than 10 cm (T2)
• larger than 10 cm but not larger than 15 cm (T3)
• larger than 15 cm (T4)
2T1
N0
M0
G2 or G3
Like 1A, except the grade is G2 and G3.
3A T2
N0
M0
G2 or G3
• The size is larger than 5 cm but not larger than 10 cm (T2).
• It has not spread to nearby lymph nodes (N0) or to distant sites (M0).
• The grade is G2 or G3.
3BT3 or T4
N0
M0
G2 or G3
Like 3A, except the size is larger than 10 cm but not larger than 15 cm (T3), or it is larger than 15 cm (T4).
3B (also)Any T
N1
M0
Any G
• The size is Any T.
• The cancer has spread to nearby lymph nodes (N1) but not to distant sites (M0).
• The grade is Any G.
4Any T
Any N
M1
Any G
• The size is Any T.
• The cancer has spread to nearby lymph nodes (N1) and to distant sites (M1).
• The grade is Any G.

What is stage 3 Ewing sarcoma?

Stage 3 Ewing sarcoma can mean that there is more than one tumor in a single bone. In soft tissues, it can instead mean that the tumor is at least 5 cm wide.

Is stage 4 Ewing sarcoma curable?

In theory, treatment should be able to cure stage 4 Ewing sarcoma. However, a 2021 paper notes that many people with stage 4 Ewing sarcoma do not respond well to treatment. Ewing sarcomas are also likely to recur after treatment.

What is the life expectancy of Ewing sarcoma?

According to the ACS, the overall 5-year relative survival rate for Ewing sarcoma is 63%. The 5-year relative survival rate is higher at 82% if the cancer is localized. If the cancer has spread to distant organs, that figure is 39%.

What does stage 4 Ewing sarcoma mean?

Stage 4 Ewing sarcoma is the most advanced stage of the condition. It means the cancer has spread to other organs and possibly distant lymph nodes.

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Ewing sarcoma is a cancer that begins in the bones the soft tissues around them. It is always a high grade cancer.

Doctors stage it by considering the tumor size, as well as its spread to lymph nodes and other organs.