Ewing’s sarcoma (ES) is a rare form of cancer that affects the bones or soft tissues surrounding the bones. Stage 4 ES is the most advanced stage of the disease. It indicates the cancer has spread to distant tissues and organs.

During the early stages of ES, a person may experience symptoms such as bone pain, swelling, and stiffness. If the disease progresses to stage 4, a person will likely experience additional symptoms, such as fever and weight loss.

At all stages of the disease, treatment typically consists of chemotherapy, surgery, and sometimes radiotherapy.

This article describes how doctors stage ES and other cancers. We also outline the symptoms of stage 4 ES and the treatment options available. Finally, we address the survival rates for stage 4 ES, the factors affecting people’s outlooks, and the recurrence rates for this type of cancer.

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The initial symptoms of ES typically include:

  • pain that may come and go, and is typically worse at night
  • stiffness
  • swelling at the site of the original tumor

This pattern of pain and discomfort may persist for weeks or months.

As the bone tumor progresses, it can weaken the bone enough to cause fractures.

If the cancer progresses to stage 4, a person may experience systemic (whole body) symptoms, such as weight loss and fever.

Doctors classify Ewing’s sarcoma (ES) and other cancers according to stages and grades.

Cancer stages

Cancer staging refers to the size of the tumor and the extent to which it has spread beyond its original location. The stages range from 0–4. Stage 0 cancers are the least advanced, and stage 4 cancers are the most advanced.

Cancer grades

Cancer grades refer to the appearance of the cancer cells under a microscope and how similar or dissimilar they look to the original cell, which indicates how quickly they grow or change.

Grades range from 1–3. Grade 1 cancers generally have a slower growth rate. Stage 3 cancers generally have a faster growth rate.

Ewing’s sarcoma stage 4

ES is an aggressive cancer that tends to spread, or “metastasize,” quickly. All ES tumors are grade 3 or “high grade” tumors. This can contribute to a stage 4 classification, which includes the following characteristics:

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

The treatment for stage 4 ES is similar to treatment for other stages of the disease. It may involve the following:

  • Chemotherapy: Chemotherapy is the standard treatment for all stages of ES. A person with stage 4 ES is likely to receive several chemotherapy drugs. However, a person who has developed recurrent ES cannot receive the same chemotherapy drugs they received previously, so this can limit their treatment options.
  • Surgery: Surgical removal of the tumor is typically the second step in ES treatment, though this depends on the location of the tumor.
  • Radiation therapy: Certain types of tumors can be difficult to treat surgically, particularly tumors in the pelvis. In such cases, doctors may use radiation therapy to destroy the cancer cells. Doctors may also use radiation therapy if prior chemotherapy or surgery has not been successful in removing all of the cancer.

A person who receives a diagnosis of stage 4 ES may also want to talk with their doctor about options to participate in clinical trials for bone marrow and stem cell transplants.

People who receive treatment for stage 4 ES may also experience a range of treatment side effects. Some examples are outlined below.

Chemotherapy side effects

Chemotherapy for stage 4 ES may cause the following side effects:

  • fatigue
  • hair loss
  • nausea
  • vomiting
  • diarrhea
  • loss of appetite
  • low levels of white blood cells, which increase the risk of infection
  • fertility issues

Radiation therapy side effects

Radiation therapy may cause the following side effects:

  • fatigue
  • skin problems
  • diarrhea
  • problems with bone growth
  • increased risk of future cancers

Effects of surgery

Some people may lose limbs as a result of surgery for stage 4 ES. Support and rehabilitation services can help people adapt to changes following surgery. A person can talk with their cancer care team for further advice.

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

For comparison, the 5-year relative survival rate during that same time frame for people with ES that had not spread beyond its original location was 82%.

Survival rates cannot predict a person’s individual outcome. They are just estimates. So many factors contribute to a person’s outlook. It is important for a person to work with their doctor to determine their outcome.

The most important factor for determining a person’s outlook with ES is whether their cancer has spread by the time they receive a diagnosis. Cancer that has spread at the time of diagnosis typically indicates a poorer outlook.

Cancer spreading into the bone marrow suggests a poorer outlook and is one of the key diagnostic criteria for stage 4 ES. A bone marrow biopsy was once the standard for determining whether cancer had spread into the bone marrow.

Currently, some researchers maintain that medical imaging, not biopsy, can provide a comparable level of information and support a classification of stage 4 ES.

According to the American Cancer Society, other factors that can affect the outlook of people living with stage 4 ES include:

  • Tumor size: Generally, the smaller the tumor at diagnosis, the better the outlook.
  • Location: Tumors located in soft tissue, further from the core, or both may indicate a better outlook.
  • LDH levels: LDH is an enzyme body tissues release in response to damage. Lower levels of LDH may indicate a more favorable outlook.

Cancer staging systems help doctors diagnose cancers, select treatment options, and discuss survival statistics. The American Joint Committee on Cancer uses the following staging system for ES and other cancers that originate in the bone or surrounding soft tissues:

  • T: describes the size and location of the main tumor
  • N: describes the extent to which the cancer has spread to nearby lymph nodes
  • M: identifies whether the cancer has spread to other organs and tissues
  • G: refers to the grade of the tumor, which medical professionals classify according to how much the cancer cells differ from normal cells

Extraosseous Ewing tumors (EOEs) are a type of ES that starts in the soft tissues surrounding the bones. The staging system for EOEs is essentially the same as for ES tumors.

Once a doctor has established the categories above, they can use these to determine the stage of the cancer.

According to a 2019 research review, 50–80% of people who have stage 4 ES at the time of diagnosis will experience a recurrence of the disease following treatment. This figure varies according to where the cancer spread.

Recurrences tend to happen fairly quickly with ES. The 2019 review notes 70% of recurrences occur within 2 years of diagnosis. For people with stage 4 ES at the time of diagnosis, the median time for relapse is 1 year.

The 2019 review further notes that around half of people with recurrent ES reported new pain or swelling, while the other half received a diagnosis of recurrent ES following a scheduled checkup.

The lungs and distant bones are the most common places for tumors to spread.

Ewing’s sarcoma (ES) is a rare cancer that affects the bones or their surrounding soft tissues. This type of cancer can grow and spread very quickly.

Cancer care teams use staging systems to diagnose cancers and guide treatment choices. Stage 4 ES is the most advanced stage of the disease. It indicates the cancer has spread to distant body organs and tissues. Symptoms may include bone pain, fractures, fever, and weight loss.

The treatment options for stage 4 ES may include chemotherapy, surgery, and radiation therapy. A person may also want to talk with their doctor about opportunities to participate in clinical trials.

People should be aware that stage 4 ES is associated with a significant risk of recurrence. As such, it is vital to get regular checkups and talk with a doctor about any recurrent or new symptoms.