What to know about Ewing sarcoma and survival rates

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ES is a rare form of cancer, affecting one out of every 1 million people in the United States. It is more common in children and adolescents, though around 30% of cases occur in people over the age of 20.

There are conflicting reports regarding whether the survival rate for ES is worse for adults than for children. A 2018 study of 397 participants with ES found that people over the age of 18 had a significantly lower survival rate compared to younger individuals.

However, a small 2017 study found that adults with ES who received chemotherapy combined with either surgery or radiation therapy had survival rates similar to those of children with ES. This suggests that aggressive, multi-pronged treatment appears to improve ES survival rates in adults.

Multiple studies maintain that survival rates for ES are better for children than for adults.

In general, 79% of children aged 0 to 14 years who receive a diagnosis of ES will be alive 5 years after their initial diagnosis.

If the cancer has not spread or “metastasized” at the time of diagnosis, the 5-year survival rate for children improves to 82%. Cancer that has spread at the time of diagnosis reduces the 5-year survival rate to 20—39%.

The most important factor affecting the prognosis for a person with ES is whether the cancer has spread or “metastasized” to other parts of the body at the time of diagnosis. The 5-year relative survival rate for individuals with ES is as follows:

• 82% for cancer that has not spread at the time of diagnosis
• 70% for cancer that has spread into nearby tissue or lymph nodes at the time of diagnosis
• 39% for cancer that has spread to distant parts of the body at the time of diagnosis

Around 1 in 4 people who have a diagnosis of ES have metastatic cancer at the time of diagnosis. Compared to adults, young children are less likely to have metastatic cancer at the time of diagnosis.

Additional factors that can affect the prognosis include:

• Tumor location: Survival rates for ES tend to be higher for individuals who have tumors located in soft tissue rather than in the bones.
• Tumor size: Survival rates tend to be higher for individuals with tumors that are smaller in volume than 100 milliliters (mL) or 200 mL.
• Levels of Lactate dehydrogenase (LDH): The body releases the enzyme LDH in response to tissue damage. High LDH levels at the time of diagnosis could indicate a large tumor or metastatic cancer, both of which are associated with reduced survival rates.
• Sex: Females tend to have a better prognosis than males.

Ewing sarcoma is potentially curable. Treatment has improved survival rates to more than 70% for people whose cancer has not spread at the time of diagnosis.

The current standard of care for ES includes chemotherapy combined with surgery, radiation therapy, or both.

Full-body or “systemic” treatment is the first-line treatment for ES and typically involves a combination of chemotherapy drugs. A person will then receive localized treatment, such as surgery or radiation therapy to destroy the tumor and any cancer cells surrounding the tumor.

Each of the above treatments can cause side effects.

Following treatment, there is a risk of ES recurring. Around 25% of people who receive treatment for localized or “nonmetastatic” ES go on to experience a recurrence of the disease following treatment.

Early relapse is common, with around 70% of relapses occurring within 2 years of the initial diagnosis.

Late relapses can also occur and typically present within 2-3 years of the initial diagnosis. Occasionally, a person may experience a late relapse 5 or more years after their initial diagnosis.

A person who has a relapse of ES may not be able to take the same chemotherapy drugs they received after their initial diagnosis, as the drugs are less likely to be effective.

As a result, healthcare professionals must search for different treatment options. The limited availability of treatment options can negatively impact survival rates.

Ewing sarcoma tends to develop in the long bones, particularly those of the lower extremities, such as the thigh and shin bones. Other common sites for Ewing sarcoma tumors to develop include:

• the pelvis, where it can be more difficult to successfully diagnose and treat
• the trunk
• the chest wall
• the upper extremities
• the spine
• the hand and foot
• the skull

In the later stages of the disease, the cancer can metastasize to the bone marrow, lungs, or soft tissue.

A person with ES may experience one or more of the following symptoms, depending on the extent of the disease:

• pain, swelling, or stiffness around the site of the tumor
• fever
• appetite loss
• weight loss
• fatigue
• numbness
• tingling
• paralysis
• incontinence

According to the American Cancer Society, the average 5-year relative survival rate for a person living with ES is around 80%. In general, the outlook appears more favorable for children than for adults.

Other factors that appear to improve the outlook for individuals with ES include:

• diagnosing and treating the cancer early, before it has had a chance to grow or metastasize
• receiving multimodal treatment, which is treatment that combines chemotherapy with surgery or radiation therapy
• not experiencing a relapse following the initial treatment

Ewing sarcoma (EW) is a rare type of cancer that primarily affects the bones or the soft tissues surrounding the bones. This type of cancer is more common among children and adolescents, though it can also develop in adults.

The current standard of treatment for ES involves chemotherapy in addition to surgery, radiation therapy, or both. Around 80% of people who receive this type of treatment are alive 5 years after their initial diagnosis.

Factors that can affect survival rates include whether the cancer has metastasized at the time of diagnosis, and whether the cancer recurs following initial treatment.

It is not clear whether a person’s age at diagnosis affects the survival rate, though most research suggests that younger individuals tend to have a better prognosis.