Stage 4 Ewing sarcoma is advanced cancer that affects the bones or soft tissue around the bones. Treatment is most effective when diagnosed early, but many factors are involved in a successful outcome.

Ewing sarcoma is a rare type of cancer. It occurs in the bones or in the soft tissue around the bones.

Typically, it begins in the leg bones or pelvic bone but can begin in any bone. It’s also possible for Ewing sarcoma to develop in the soft tissue of the abdomen, chest, or lungs.

People of any age can get this cancer, but it’s most common in people under age 20.

Stage 4 Ewing sarcoma is advanced cancer. In stage 4, Ewing sarcoma has spread to the lymph nodes and to areas of the body beyond the original cancer site.

Treatment and positive outcomes at this stage are still possible, though the best outcomes occur when it’s diagnosed early. Individual factors can play a big role in the treatment success for Ewing sarcoma.

Ewing sarcoma is a rare type of cancer. People of any age can develop this cancer, but it’s most often seen in children and young adults.

Ewing sarcoma is the second most common type of bone cancer in children. It makes up about 2% of childhood cancer diagnoses.

According to the National Organization for Rare Disorders (NORD), 2.93 out of every 1 million Americans between 10–20 years old develop Ewing sarcoma.

The cancer is slightly more common in males and is more often seen in people of European heritage than people of African or Asian heritage.

Ewing sarcoma can be diagnosed at any stage. Often, it’s discovered when a parent brings a child to the doctor for what seems to be an injury. In other cases, an actual injury leads to a diagnosis of Ewing sarcoma when X-rays spot the tumor.

However, Ewing sarcoma progresses quickly and early symptoms can be easy to miss. Some children and young adults are first diagnosed at stage 4.

A cancer stage refers to the size of the tumor and how much the cancer has spread past its original location.

Cancer stages range from 0 to 4. Stage 0 cancers are small tumors that haven’t spread at all, while stage 4 cancers have spread to distant areas of the body and the tumors are larger.

In stage 4 Ewing sarcoma, it has most often spread to the lungs, other bones, or bone marrow.

Ewing sarcoma is a fast-growing sarcoma. It can progress through stages very rapidly.

Stage 4 Ewing sarcoma means:

  • there is more than one tumor in a bone
  • it has spread to nearby lymph nodes
  • it has spread to organs such as the liver, lungs, or brain
  • the cancer has spread to distant parts of the body

Stage 4 Ewing sarcoma treatment focuses on killing cancer cells and removing or reducing the size of the tumor. Treatments are similar to treatments used at all stages of Ewing sarcoma.

Treatments typically involve:

  • Chemotherapy: Chemotherapy is an important part of treatment at all stages of Ewing sarcoma. People with stage 4 Ewing sarcoma typically receive very potent drug combinations.
  • Surgery: Surgery to remove the tumor will be done if possible. Doctors will look at the size and location of the tumor to see if this is an option.
  • Radiation therapy: Radiation therapy is often used when surgery cannot be done. Radiation can destroy cancer cells and tumors.
  • Clinical trials: People with stage 4 Ewing sarcoma might be eligible for clinical trials. Your doctor can let you know what options may be available to you.

What can affect treatment outcomes?

There are several factors that contribute to treatment success in stage 4. The biggest factor is how far cancer has spread.

Although any cancer classed stage 4 will have spread, some cases will have spread much further into the body and organs than others.

As a general rule, treatment is less successful when cancer has spread throughout the body. Additionally, the specific places the cancer spreads can have an impact. According to 2017 research, outcomes are typically better if cancer does not spread into the bone marrow.

However, it’s important to keep in mind that individual factors are also important. A person’s age, overall health, genetics, and response to treatments can all have a large influence on outcomes. It’s impossible to predict an exact disease course just by looking at the cancer stage or tumor size.

According to the American Cancer Society, the 5-year survival rate for people with stage 4 Ewing sarcoma was 39% between 2010 and 2016.

The 5-year survival rate for people with Ewing sarcoma who were diagnosed early was 82% during that same period.

Survival rates are based on past data. Cancer treatments have improved in the past decade. It’s possible that current survival rates are higher than these numbers suggest. Additionally, these numbers are only estimates and cannot predict individual outcomes.

The exact symptoms of Ewing sarcoma depend on which bone is affected. However, by stage 4, the cancer will have metastasized, or spread. This means it’s likely that symptoms will also be widespread.

Stage 4 Ewing sarcoma symptoms might include:

  • whole-body weakness
  • weight loss
  • fever
  • fatigue
  • fragile bones

In earlier stages, symptoms can sometimes be easier to miss. They might be mistaken for simply being worn down or for a minor injury.

Early symptoms can include:

  • stiffness
  • pain that is worse at night
  • swelling
  • pain that comes and goes but doesn’t get better

Ewing sarcoma is a rare bone tumor that’s most common in people under age 20. The tumor is fast growing and symptoms can be easy to miss at early stages.

People diagnosed with stage 4 Ewing sarcoma might notice symptoms such as weakness, fever, unintentional weight loss, and fragile bones.

In stage 4, the first treatment is chemotherapy to kill cancer cells. In many cases, the second step is surgery. However, surgery isn’t always possible. The size and location of the cancer will determine if surgery is an option. If it isn’t, radiation therapy will be used.

Stage 4 Ewing sarcoma is treatable. The size and spread of the tumor, as well as individual factors, can play a big role in the outcome.