Metastatic Ewing’s Sarcoma

Metastatic Ewing’s sarcoma is a challenging condition in which cancer that began in bone or soft tissue has spread to distant parts of the body, most commonly the lungs, presenting unique treatment challenges and requiring aggressive therapy approaches.

Table of contents

• What Is Metastatic Ewing’s Sarcoma
• How the Cancer Spreads
• Outlook and Survival
• Treatment Approaches
• Chemotherapy for Metastatic Disease
• Local Treatment Options

What Is Metastatic Ewing’s Sarcoma

Metastatic Ewing’s sarcoma refers to cancer that has spread from the bone or soft tissue where it originally started to one or more distant sites in the body. This spread, called metastasis, means the cancer cells have traveled beyond the initial location to other parts of the body^[1].

About 25% of patients with Ewing’s sarcoma present with visible metastatic disease at the time of diagnosis. However, an even larger number of patients have hidden microscopic disease throughout their body at baseline, even when tests don’t show it^[5].

Ewing’s sarcoma is classified in a simple way: either as localized disease, meaning the tumor has not spread beyond where it began or the closest surrounding tissues, or as metastatic disease, meaning the tumor has spread to distant locations^[7].

How the Cancer Spreads

The most common place for Ewing’s sarcoma to spread is the lungs. When the cancer reaches the lungs, patients typically have a better chance of recovery compared to patients whose cancer spreads to other distant locations^[6].

Ewing’s sarcoma can also spread to other bones or to the bone marrow, which is the soft tissue inside bones where blood cells are made. When cancer spreads to these areas, it becomes more difficult to control^[7].

This cancer has an aggressive nature with a high tendency to spread throughout the body. Because of this behavior, about 80% to 90% of patients have widespread microscopic disease at diagnosis, even when imaging tests appear normal^[5].

Outlook and Survival

The outlook for patients with metastatic Ewing’s sarcoma is generally more serious than for those with localized disease. Despite aggressive treatment with chemotherapy and local therapy, long-term cure is achieved in only 20% to 30% of patients with upfront metastatic disease^[5].

The location where the cancer spreads matters significantly. Patients who have cancer that spreads only to the lungs have better outcomes than patients whose cancer spreads to bones or other locations^[6].

Over the past 40 years, both local therapy and combination chemotherapy have led to remarkable improvements in treating localized Ewing’s sarcoma, with survival rates increasing from less than 20% to greater than 70%. However, the outlook for metastatic disease remains challenging, and the rate of cancer coming back remains high^[3].

Treatment Approaches

Treating metastatic Ewing’s sarcoma requires a team approach. The treatment team typically includes the primary care doctor, an orthopedic surgeon who specializes in bone tumors, a specialist who examines tissue samples to identify disease, radiation specialists, cancer doctors who use medications, rehabilitation specialists, specialized nurses, social workers, and others. An experienced team is best found in specialty cancer centers that treat many patients with Ewing’s sarcoma^[6].

Treatment for metastatic Ewing’s sarcoma typically involves a combination of approaches, which may include surgery, radiation therapy, chemotherapy, or all three methods. This multi-method approach is increasingly recognized as important for increasing a patient’s chance of cure or prolonging survival^[6].

Because patients with metastatic Ewing’s sarcoma have disease that has spread to various parts of the body, treatment that affects the whole body is necessary. In many cases, it may also be necessary to treat the original tumor with surgery, with or without radiation therapy, for the best results^[6].

Chemotherapy for Metastatic Disease

Chemotherapy uses medications to kill cancer cells throughout the body. The standard chemotherapy treatment combines several drugs. The most common combination includes medicines called Adriamycin (doxorubicin), Oncovin (vincristine), Cytoxan (cyclophosphamide), and Actinomycin D (dactinomycin)^[6].

Many doctors also use a treatment plan that alternates between two different drug combinations. This approach includes Oncovin, Adriamycin, and Cytoxan, which then switches with Ifex (ifosfamide) and Vepesid (etoposide)^[6].

Treatment typically lasts 6 to 9 months. Patients receive alternating cycles of two different chemotherapy combinations. Chemotherapy can be given either in the hospital or as an outpatient, depending on how well the patient tolerates treatment and how close they live to the hospital^[12].

Patients often develop fevers while their white blood cell counts are low between treatment cycles, which may require hospital stays lasting 3 to 7 days^[12].

Local Treatment Options

Managing the site where the tumor originally started is critical to long-term cure. When possible, surgery to completely remove the tumor with clear margins around it is the preferred approach. This might include removing certain bones like the fibula or performing a procedure to save the limb while removing the tumor with wide margins. Any surgery should be performed by experienced cancer surgeons who specialize in the area of the body where the tumor is located^[12].

When surgery would cause significant problems or disability, radiation therapy can be used to control the local tumor. The amount of radiation and how it is delivered depends on where the tumor is located and the specific characteristics of each patient’s situation^[12].

In some instances, the chance of cure or relief of symptoms can be improved by treating metastatic tumors with surgery or radiation therapy after chemotherapy has been given^[6].