Ewing's Sarcoma Treatment
The experts at MD Anderson's Children's Cancer Hospital take the extra time and effort to personalize treatment for each child with Ewing's sarcoma. A highly specialized team of some of the world's top physicians follows your child throughout treatment, ensuring the best chance of successful treatment with the least impact on your child's growing body.
Ewing's sarcoma is rare, and most oncologists have little experience treating it. Studies show that sarcoma patients have better outcomes when they are treated at large, comprehensive cancer centers. Our physicians treat a large number of patients with Ewing's sarcoma, which translates to a remarkable level of skill and expertise.
Surgical expertise
Since Ewing's sarcoma usually requires multiple approaches, a comprehensive team approach is crucial. At the Children's Cancer Hospital, your child is the focus of a group of physicians, including surgeons, oncologists, radiation oncologists and pathologists. They are supported by specially trained rehabilitation specialists, therapists, nurses and other health care professionals who work together to ensure your child receives the best care.
Ewing's sarcoma treatment often includes surgery. Our surgeons are among the nation's most skilled. They use the most up-to-date techniques and technologies, including limb-sparing surgeries, so most patients can avoid the loss of an arm or leg. If limb-sparing surgery is not an option, Children's Cancer Center offers the most-advanced prostheses.
Leading-edge Ewing's sarcoma research
Many Ewing's sarcoma patients at Children's Cancer Hospital are treated with innovative agents as part of clinical trials. We participate in Children's Oncology Group protocols, as well as other clinical trials available at only a few hospitals in the nation.
If your child has been diagnosed with Ewing's sarcoma, we're here to help. Call 877-632-6789 to make an appointment or request an appointment online.
Our Ewing's sarcoma treatments
If your child is diagnosed with Ewing's sarcoma, the doctor will talk to your family about the best options to treat it. This depends on several factors, including the size and location of the cancer, if it has spread, and your child's age and general health.
At Children's Cancer Hospital, we design a course of treatment customized specially for your child's needs. One or more of the following therapies may be recommended to treat the cancer or help relieve symptoms.
Chemotherapy
In most Ewing's sarcoma cases, small areas of cancer have spread to other parts of the body. They are called micrometastases. These areas usually do not show up in imaging tests.
For that reason chemotherapy, which travels to all parts of the body, is almost always the first part of the treatment for Ewing's sarcoma. Usually, it is followed by surgery and/or radiation therapy and further chemotherapy. Children's Cancer Hospital offers the most up-to-date and advanced chemotherapy options.
Targeted therapies
Children's Cancer Hospital is leading into the future of cancer treatment by developing innovative targeted therapies. These agents are specially designed to treat each cancer's specific genetic/molecular profile to help the body fight the disease. Many of the doctors who treat Ewing's sarcoma at Children's Cancer Hospital are dedicated researchers who have pioneered and actively lead national and international clinical trials with novel targeted agents.
Surgery
Because Ewing's sarcoma can be found in a number of places throughout the body, several types of surgery may be used to treat it. Your doctor will talk to you about the best type of surgery for your child's particular needs and possible side effects.
Surgery to treat Ewing's sarcoma attempts to remove as much of the tumor as possible without harming surrounding areas.
At Children's Cancer Hospital, our specialized orthopedic surgeons are often able to avoid amputation when the tumor is in an arm or leg. Complex limb-sparing surgery removes the tumor, but saves the tendons, nerves and blood vessels. Any bone that is removed is replaced with a bone graft or an internal prosthesis, which is similar to an artificial joint.
Like all surgeries, Ewing's sarcoma surgery is most successful when performed by a specialist with a great deal of experience in the particular procedure. Children's Cancer Hospital surgeons are some of the most skilled and recognized in the world. They are among the few surgeons in the world who specialize in pediatric sarcoma treatment, and they use the least-invasive and most-advanced techniques designed especially for children.
Radiation therapy
Radiation therapy (also called radiotherapy) uses high-energy beams to destroy cancer cells. New radiation therapy techniques and remarkable skill allow Children's Cancer Hospital doctors to target tumors more precisely, delivering the maximum amount of radiation with the least damage to healthy cells. This is particularly important to growing bodies.
Children's Cancer Hospital provides the most-advanced radiation treatments, including:
- 3D-conformal radiation therapy
- Intensity-modulated radiotherapy
Proton therapy
Proton therapy delivers high radiation doses directly into the tumor, sparing nearby healthy tissue and vital organs. For many patients, this results in better cancer control with less impact on the body.
The Proton Therapy Center at MD Anderson is one of the world's largest and most advanced centers. It's the only proton therapy facility in the country located within a comprehensive cancer center. This means this cutting-edge therapy is backed by all the expertise and compassionate care for which MD Anderson is famous.
Learn more about ewing's sarcoma:
Newest research in sarcoma
Sarcomas?are a broad group of cancers that originate from connective tissues, such as the blood or lymph vessels, gastrointestinal tract, fat tissue, tendons, smooth muscles and tissue around the joints. These tumors are broadly divided into?bone sarcomas?and?soft tissue sarcomas. With more than 70 subtypes of sarcoma, there are also a wide variety of treatment approaches and prognoses, and everything about the disease, from diagnosis onward, is complex.
¡°If we look back over the last decade, we¡¯ve made some tremendous improvements,¡± says?, associate professor of?Sarcoma Medical Oncology. ¡°However, there is still more we can do to improve therapeutics for sarcoma.¡±
Conley and his colleagues will present some of these new approaches at the 2024?American Society of Clinical Oncology (ASCO) Annual Meeting.
A virus to help the immune system attack tumors
Conley¡¯s oral presentation () is about the results of a multicenter?clinical trial?using a genetically modified adenovirus combined with an immune checkpoint inhibitor to treat sarcoma, along with several other cancer types, that didn¡¯t respond to immunotherapy alone. ¡°There needs to be something else to invigorate the immune system to help fight the cancer,¡± he says.
Adenoviruses cause the common cold, but this one, called AdAPT-001, is genetically modified to help cause inflammation in the cancerous tumor, which then attracts the immune system to attack. It also changes the environment around the tumor to make it more responsive to immunotherapy. ¡°It does that by introducing what is called TGF-¦Â trap,¡± Conley says. ¡°The virus has been engineered to soak up different forms of TGF-¦Â. This then allows your T-cells, which are an important part of the immune system, to now activate, recruit other cells and damage the tumors.¡±
Side effects from AdAPT-001 were generally mild.
Conley and his team found that injecting AdAPT-001 directly into one or more tumors and also giving the patient an immune checkpoint inhibitor had a clinical benefit rate of 62.5% at 12 weeks. ¡°It was a small study, but we found some really interesting results,¡± he says. ¡°For example, in several cutaneous sarcomas treated with the combination therapy, the tumors visually looked better in scans ¡ª smaller and less irregular.¡± Furthermore, tumors that were not directly injected were improving as well.
In patients treated previously with immune checkpoint inhibitors, the combination treatment with AdAPT-001 allowed them once again to see a response.
An earlier part of this study was?among the presentations related to sarcoma at last year¡¯s ASCO annual meeting.
Risk stratifying sarcoma patients
Ryan Denu M.D., Ph.D., a second-year medical oncology fellow who was recognized with both the Conquer Cancer Merit Award and the 2024 ASCO Young Investigator Award, will present research () he completed under the supervision of?, assistant professor of Sarcoma Medical Oncology. ¡°He took a gene he was interested in studying in the lab, ATRX, and wanted to find out what it could tell us about treatment or even about prognosis in a variety of soft tissue sarcomas,¡± Conley says.
The ATRX gene is involved with??and acts as a regulator of transcription, meaning the process of creating an RNA copy of a DNA sequence. Denu¡¯s research indicates that a number of different sarcomas have a loss of ATRX gene expression and function. ¡°We think this may be essential to the development of the cancer and may also have prognostic value,¡± Conley says.
Denu correlated the presence of the ATRX gene and the protein it codes for with different aspects of the immune system. It seems that without the gene, the tumor is better able to avoid contact with the immune system and protect itself from the body¡¯s defenses. Therefore, loss of ATRX is associated with both a distinct type of microenvironment?¡ª one that suppresses the immune system around the tumor?¡ª and worse overall survival.
¡°This has important implications for us as a way to potentially determine which patients might benefit from certain types of immunotherapy,¡± Conley says. ¡°If this is validated in further studies, it might be something that we will routinely test for in a clinical setting.¡±
Potential for a blood test to predict outcomes
, division head of Pediatrics and interim department chair of Sarcoma Medical Oncology, was the middle author of research () about patients with metastatic Ewing sarcoma that were treated in the Children¡¯s Oncology Group trial. Although Ewing sarcoma is commonly thought of as a disease affecting children, adolescents and young adults can also be diagnosed. The research found an association between increased levels of circulating tumor DNA (ctDNA) in the bloodstream following one cycle of chemotherapy and inferior outcomes.
¡°It might lead to people getting routine blood tests to look for ctDNA levels while they are being treated for Ewing sarcoma,¡± Conley says.
Gorlick, recipient of the ASCO 2024 Pediatric Oncology Award, will also be a discussant on the sarcoma oral abstract session as ASCO.
Poster presentations also highlight sarcoma research
, associate professor of Sarcoma Medical Oncology and both an adult medical oncologist and a pediatrician, is presenting a ¡°trials in progress¡± poster () about a T cell membrane-anchored tumor-targeted IL12 (ATTIL12) -T cell therapy for sarcoma. ¡°It is a cool and innovative study,¡± Conley says. ¡°MD Anderson is the only place in the world that has this technology, which was developed by the pediatric oncology group.¡±
Alicia Gingrich, M.D., a complex general surgical oncology fellow, will be presenting a poster () about the immune microenvironment of leiomyosarcomas, which are smooth muscle sarcomas. Under the direction of Nassif Haddad, she looked at tissue samples of leiomyosarcomas under a microscope to find common characteristics and try to determine which patients need to be considered for immunotherapy and which immunotherapy treatments would be best for them. ¡°This is great work that will hopefully lead to clinicians being better able to tailor treatments to the individual patients,¡± Conley says.
What¡¯s next in sarcoma research
Sarcoma research at MD Anderson goes beyond these presentations at ASCO.
For example,?, professor of Sarcoma Medical Oncology, and her colleagues recently??of a clinical trial of the first T cell therapy for patients with metastatic or unresectable synovial sarcoma or myxoid round cell liposarcoma. These patients had few options if the standard treatment of high-dose chemotherapy didn¡¯t work. With the T-cell receptor therapy, many had durable responses, meaning their cancer responded to the treatment for at least six months and possibly longer, as the follow-up of these patients is still ongoing.
In addition, new research led by?, associate professor of?Surgical Oncology?and?., associate professor of?Sarcoma Medical Oncology, has found that?immunotherapy before surgery leads to promising long-term survival in sarcoma patients.
¡°Our department has moved toward participating in multi-center clinical trials and leading clinical trials,¡± Conley says. ¡°We are also very interested in incorporating immunotherapy into our treatments for sarcomas or even replacing some of the therapies we once used to treat these diseases. It¡¯s an exciting time.¡±
?at MD Anderson.
Ewing's sarcoma is treated in our Children's Cancer Hospital, Sarcoma and Orthopaedic Center and our Proton Therapy Center
Ewing¡¯s sarcoma caregiver: Finding a targeted radiation plan for my son
In Sept. 2019, I discovered a rock-hard bump on my son Logan¡¯s chest. The next day, an X-ray revealed a soft tissue mass on his chest. A CT scan showed two additional masses on his chest. By Oct. 2019, a biopsy confirmed a Ewing¡¯s sarcoma diagnosis.
Logan did not show the genetic markers of Ewing¡¯s sarcoma, but he had several gene mutations. I started researching as much as I could. I wanted to know everything I could about his diagnosis.
A week later, Logan began six rounds of chemotherapy at our local hospital. He handled chemotherapy very well. But he experienced side effects, including neutropenia, mouth soreness and fatigue.?
In Jan. 2020, Logan underwent a full thoracotomy to remove the tumors from his diaphragm. During surgery, doctors discovered multiple nodules throughout his chest cavity. They performed a partial pleurectomy, along with an ablation of the visible nodules. After surgery, he was up and walking by the fifth day. And by the seventh day, he was off pain medication.
In March 2020, he underwent another surgery for a chest wall resection to remove three partial ribs. Then he received a full pleurectomy of the remaining pleura of his chest wall. Logan was amazing and kept a positive attitude. After surgery, he said, "This surgery was much easier than the last!"
Choosing targeted radiation therapy at MD Anderson
After speaking with multiple specialists, we knew it was vital for Logan to receive radiation to the tumor sites. Since the primary tumor was on his rib and several other tumors had been attached to his diaphragm, we needed a targeted radiation plan. It was important to keep his healthy tissues and organs unharmed.
That¡¯s when we met at the MD Anderson Proton Therapy Center. Dr. Paulino took every approach to ensure that Logan¡¯s radiation treatment plan would be the best overall.
Starting on May 12, Logan received 23 treatments of volumetric modulated arc therapy (VMAT) to his hemithorax. That was followed by seven proton therapy treatments. To minimize skin irritations from the radiation, I applied aloe vera infused with manuka honey, along with heavy amounts of ointment, to his skin. He also took a shark liver oil supplement to help reduce other side effects from radiation, such as nausea and to improve his immune system.
Logan¡¯s radiation therapists made sure he was comfortable. They always greeted him with encouraging words and got to know him. They asked him questions to help him open up. Logan played video games and created animations to redirect his focus.
In Sept. 2020, we found out Logan is cancer-free. His lungs look great and show no damage from radiation. He can exercise and keep active like any other 10-year-old boy.
Coping with your child¡¯s cancer treatment
My advice to other parents is to ask questions. Always look for ways to help your child through the process.?As scary as it is to have a child with cancer, it is even scarier when issues arise that you are not aware of.?
Communication with your child during treatment is key. Keep a care kit available for needs such as sores, rash, open wounds, fatigue or nausea. Stay prepared by speaking with your care team regularly to identify possible situations that could arise.???
Try to keep your spirits up. We joke and laugh a lot. As hard as life is right now with quarantine, it¡¯s about being together with family, and embracing the positivity that life has to offer.
or by calling 1-877-632-6789.
3 things I learned during Ewing¡¯s sarcoma treatment
In November 2016, when I was 9 years old, I was diagnosed with Ewing¡¯s sarcoma. My parents took me to MD Anderson, where I had chemotherapy, surgery and radiation therapy. I also got daily injections for 11 months, to keep me from developing blood clots.
I¡¯m glad that all of those treatments worked, because I haven¡¯t shown any signs of cancer since my surgery in February 2017. I still had to finish my treatments after surgery to make sure the Ewing¡¯s sarcoma was completely gone, but I learned a few things during those 10 months.
Here are three of them.
You can get used to anything
Of all the treatments I received, the Lovenox injections worried me most. Those were a shot in my stomach every day ¡ª and sometimes twice a day ¡ª for months and months. The chemotherapy didn¡¯t really hurt or scare me, but it did make me lose all my hair.
The radiation therapy didn¡¯t hurt either, but they made me feel really sick. And when I saw the radiation machine for the first time, I thought it looked like a giant donut that was going to eat me. So as silly as it sounds now, I have to admit, I was a little afraid of it.
Over time, though, I got used to everything. To deal with losing my hair, I found fun hair garments to wear. I learned how to handle the IVs while getting chemotherapy. And I even learned how to sleep with a bandage wrapped around my middle from the radiation burns. I had lots of peeling skin, and it was always itchy. But after a while, there wasn¡¯t much that felt scary anymore.
You can always find happiness
The worst part of my Ewing¡¯s sarcoma treatment was being attached to an IV pole when I was an inpatient for chemo treatments. I just felt really sick all the time and I hated it. I would lay down on the cold tile floor in the bathroom sometimes and just cry, because I hated throwing up so much.
But if you have a positive attitude as much as you can, your treatment will go faster. So, even if you don¡¯t want to be around anyone, try some of the games, like bingo, or watch movies with other patients and families. Do any of the activities that MD Anderson offers, if you¡¯re not feeling too sick. It will help distract you from what¡¯s going on, and make the time pass faster.
Also, try to set aside the bad things after they¡¯ve happened and enjoy the good things in your life. Because sulking and being sad about having cancer doesn¡¯t help at all. And there¡¯s almost always something to smile about. I got free food and special treats (like ice cream!) whenever I wanted them. And when I missed a friend¡¯s sleepover birthday party due to treatment, my Mom and I had a party of our own. We pretended we were celebrating our own birthdays. We did each other¡¯s makeup, watched movies, ordered Thai food and ate s¡¯mores.
I can do anything
I¡¯m still healing in my stomach area from the surgery and radiation, so I have to be careful in PE if we do certain activities. I also have to be more careful about germs, washing my hands a lot and trying not to be near people who are sick. But mostly, I feel pretty good. I¡¯m starting to get my energy back and enjoy playing outside again.
Before I got sick, I didn¡¯t give much thought to my future, but now I¡¯m feeling more excited about it. I feel like I can accomplish more, like my dream of being an actress. My first dream was getting through my cancer treatment. Now that that¡¯s finished, I feel unstoppable, like I can do anything.
or by calling 1-877-632-6789.
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Progress in Ewing¡¯s sarcoma treatment
Ewing¡¯s sarcoma is a rare cancer that can occur in the bones or soft tissues. It¡¯s more common in children and teens than in adults, but it accounts for only around 1% of childhood cancer cases. Only about 30% of patients with Ewing¡¯s sarcoma are over age 20, and the disease tends to be even more difficult to treat in these cases.
Although many patients with Ewing¡¯s sarcoma are cured with traditional chemotherapy, there haven¡¯t been many treatment options for patients with relapsed disease. But recently, researchers have discovered new drugs and therapeutic regimens that may provide more treatment options for Ewing¡¯s sarcoma patients.
We spoke with , assistant professor of Sarcoma Medical Oncology,?to learn about advances in sarcoma treatment, including results from a clinical trial he is presenting at the virtual ().
How is Ewing¡¯s sarcoma typically treated?
In children and adolescents, oncologists typically prescribe a combination of five drugs given in an alternating order for up to a year. This combination therapy is able to cure around 70% of young patients with localized disease. But if the cancer is metastatic, does not respond to treatment, or relapses, then prognosis tends to be poor. ?
Variations of this combination therapy are used for adults, as well, but often adults cannot tolerate a full year of the same intensive treatment given to children. This may partially explain why adult patients with Ewing¡¯s sarcoma have lower rates of cure than younger patients. Therefore, our researchers are trying to find tolerable and effective treatments for adult patients with Ewing¡¯s sarcoma.
How do genetic markers influence development of Ewing¡¯s sarcoma?
Ewing¡¯s sarcoma is one of several sarcomas that are caused by specific genetic changes. The most common genetic change with this disease is fusion of two genes called EWSR1 and FLI1. FLI1 is part of a group called ETS genes, and when EWSR1 fuses with FLI1, an abnormal transcription factor protein is created. This protein then interacts with DNA in a way that causes cells to grow abnormally and develop cancer in the bone or soft tissue.
What research is being done on therapies that target the genetic markers of Ewing¡¯s sarcoma?
In our ongoing clinical trial including adult and pediatric patients with metastatic, relapsed or refractory Ewing¡¯s sarcoma, we are administering the standard chemotherapy drug vincristine in combination with TK216, a novel inhibitor of ETS proteins. It can be difficult to target transcription factors like the abnormal one created by the EWSR1-FLI1 fusion, but TK216 seems to work by interrupting the interactions between this abnormal protein and the genes that drive the cancer¡¯s growth.
Overall, almost half of the patients treated with TK216 experienced clinical benefits ¨C either complete response or stable disease ¨C and two patients are now in complete remission with no evidence of recurrence for over one and two years. The drug is well-tolerated, with common side effects being fatigue and low red or white blood cell counts. We believe combination therapy with TK216 is a promising option for the subset of patients with metastatic and relapsed Ewing¡¯s sarcoma.
What future research is needed?
While we see a number of patients who clearly benefit from TK-216, others do not. At this time, we do not know why that is. We need to further examine the relationship between new drugs like this and the specific properties of a given patient¡¯s cancer so that we can determine, before we start treatment, which patients are likely to respond to a therapy and which will not. Although Ewing¡¯s sarcoma is largely driven by a single common genetic change, each case progresses differently. We don¡¯t understand all of the factors that contribute to a patient¡¯s response to treatment.
For TK-216 in particular, we also understand from laboratory studies that prolonged treatment seems to be more effective than short treatments. This is why the medication in the study is given continuously via an infusion pump for 14 days. Finding ways to make even longer treatment courses more manageable with dose alterations or better supportive treatment may be helpful in increasing the benefit patients obtain from the medication.
or by calling 1-877-632-6789.
?
Ewing¡¯s sarcoma is a rare cancer that can occur in the bones or soft tissues. It¡¯s more common in children and teens than in adults, but it accounts for only around 1% of childhood cancer cases. Only about 30% of patients with Ewing¡¯s sarcoma are over age 20, and the disease tends to be even more difficult to treat in these cases.