Get the Facts on Ewing’s Sarcoma Now
Ewing’s sarcoma is a cancerous tumor that develops in bone. It is named after Dr. James Ewing who first described it in 1921. ES is considered to be a rare type of cancer, affecting only about 1 out of 100,000 children. However, recent studies have shown that its incidence rate may actually be higher than previously estimated. There are two types of Ewing’s sarcoma: extraosseous (ES) and peripheral primitive neuroectodermal tumors (PNET). ES occurs in soft tissue while PNET occurs in bone. Both forms occur equally often in children and young adults. Studies are investigating treatments for ES including chemotherapy, radiation therapy, immunotherapies, and stem cell transplants. More research is still needed to improve existing therapies and identify new methods for treating Ewing’s Sarcomata. According to the American Cancer Society, approximately 2,600 new cases of ES were reported in the United States in 2017.
All types of ES affect the white blood cells called lymphocytes. Lymphocytes help protect your body from disease. If a tumor begins to spread beyond its initial site, lymphocytes attack the tumor and destroy it. However, when a tumor gets bigger, more and larger numbers of lymphocytes are able to join together in attacking the tumor. Over time, this makes the tumor harder to treat. That’s why treatment often takes longer to work.
In addition to destroying the tumor, lymphocytes also fight infection in your body. Because lymphocytes are so important in fighting infection, patients who get chemotherapy or radiation therapy to shrink a tumor often experience severe fatigue after being treated. People with leukemia often receive blood transfusions to increase the number of blood cells making them stronger.
ES is very sensitive to radiation treatments. Radiation kills cells, including lymphocytes. Patients receiving radiation treatments are at high risk of losing the ability to make lymphocyte counts go back up. This means the ability to fight infections becomes weak.
Treatment for Ewing’s Sarcoma: If surgery doesn’t remove enough of the tumor, doctors use radiation therapy. Doctors use different doses of X-rays delivered either daily or weekly as a radiation treatment. Usually, this combination lasts four to six weeks. In some cases, patients need even higher doses of radiation. Surgery followed by radiation is often effective but isn’t always possible. Radiation can cause serious side effects.
Chemotherapy works by killing fast-dividing cells like those in tumor cells. Chemotherapies kill both good and bad cells, not just tumor cells. Unfortunately, chemo drugs don’t last forever. After the first round of treatment, patients must wait months between each dose until their bodies recover enough to take the next shot.
Patients who do well usually receive a drug cocktail. One can take Drugs intravenously or orally. Oral medications are taken twice a day while IV drugs are administered three times a week. Combinations of drugs are given, depending on the type and size of the tumor. While the exact mix varies from person to person, many combinations include vincristine, cyclophosphamide, doxorubicin, etoposide, prednisone, and/or bleomycin.
Unfortunately, chemotherapy alone won’t cure ES. About half of people survive five years without further treatment. Survival rates drop sharply after that. Even though some studies show survival rates of 60% or more for patients who receive intensive therapy, others find much lower results.
Because the treatment options are so poor, scientists are researching ways to improve the current treatments. One promising option involves giving certain kinds of targeted therapies that only target specific parts of a tumor. For example, patients might receive radiation to shrink an area of the tumor. Then researchers could give a drug that targets the remaining portions of the tumor. This kind of approach might allow patients to receive fewer treatments and still achieve similar results.
Many people with Ewing’s sarcomas have been cured by using aggressive treatments early in the disease. But research shows that many people diagnosed now will die instead of surviving. And many survivors face significant complications.
How do Ewing’s Sarcomas develop?
In order to understand how ES develops, it helps to know the basics about the normal development of the body. Two different types of cells makes bone. Osteoblasts make hard, dense connective tissue called bone matrix. These osteoblasts are what help build up the skeleton. When bone is broken down, osteoclasts remove the old bone while forming the new bone matrix.
What causes Ewing’s Sarcomatous tumors?
When cancer cells begin dividing uncontrollably, they create abnormal numbers of chromosomes. This makes them much more likely to become malignant than they would if their chromosomes were balanced. As these cancers continue to grow, they may invade nearby soft tissue. If cancer grows past the lining of the bone cavity, it can spread throughout the body.
The diagnosis of Ewing’S Sarcoma begins with a physical exam and imaging tests. A biopsy confirms the diagnosis. Imaging tests include X-rays, CT scans, MRIs, PET scans, and bone scans. These tests help identify if the tumor is located in the bones, lungs, lymph nodes, liver, or elsewhere.
Patients treated with non-operative methods have a survival rate of approximately 80%. Patients treated with surgical procedures have a five-year survival rate of 50%.
There are no known risk factors for developing Ewing’s Sarcoma. However, some studies have shown that people who play sports may be at increased risk. Other possible risk factors include exposure to radiation, certain chemicals, and genetic mutations. Children ages 5–14 years old are most likely to get Ewing’s Sarcomatosa. Boys are slightly more likely than girls to get Ewing’s sarcomatoid. Children who live in rural areas are more likely to develop Ewing’s sarcoidosis than those who live in urban areas.
Symptoms of Ewing’s Sarcoma
The symptoms of Ewing’s Sarcoma vary depending on where the tumor is located. In general, they include:
- Pain in bones
- Swelling in joints
- Weight loss
- Difficulty breathing
- Abdominal pain
- Surgery: It is best to perform surgery in the beginning. However, if there is no good margin around the tumor, then amputation may be necessary. Following the removal of the tumor, additional surgeries help improve limb function.
- Chemotherapy: You can take Chemotherapeutic drugs before, during, or after surgery.
- Radiation Therapy: It is common to prescribe radiotherapy and chemotherapy together in the treatment.
- Targeted Therapies: Drugs like tyrosine kinase inhibitors specifically target abnormal cells. These drugs block the activity of enzymes, including protein kinases, involved in regulating cell growth and division.
- Immunotherapy: Monoclonal antibody tests are the potential treatments for ES patients. These antibodies bind to specific molecules on the surface of tumor cells, triggering the body’s immune system to attack them.
- Stem Cell Transplantation: Bone marrow transplantation involves replacing the patient’s own blood-forming stem cells with those donated by a sibling or unrelated donor.
What happens if Ewing’s sarcomatous doesn’t respond to treatment?
If the tumor doesn’t shrink after treatment, doctors may try different combinations of treatments until they find the best plan for each person. Sometimes, patients need additional treatment even after the initial treatment fails.
Is Ewing’s sarcoma curable?
Cure rates depend on many factors including the type of treatment, stage of disease, location of the tumor, and patient’s general condition. Treatment options include surgery, chemotherapy, radiation therapy, and/or targeted therapies.