What’s New in Ewing Tumor Research and Treatment?

Research on Ewing tumors is being done at many medical centers, university hospitals, and other institutions across the world.

Understanding and diagnosing Ewing tumors

Scientists are developing new techniques to more accurately diagnose Ewing tumors. New lab tests of tumor samples are being studied to see if they can help identify Ewing tumors and give more information on how well treatments might cure that particular tumor.

As researchers learn more about the changes inside Ewing tumor cells that make them different from normal cells, they hope to develop new treatments that take advantage of these changes (see below).


Researchers are looking to develop better treatments for Ewing tumors, as well as to find less toxic treatments for those that can be cured.

Radiation therapy

Ewing tumors are very sensitive to radiation therapy, but because of its possible side effects, it's most often used only if surgery can't be done for some reason. Newer, more focused types of radiation therapy can help doctors treat tumors while lowering the dose of radiation to nearby healthy body tissues.


The Children’s Oncology Group, as well as many cancer centers and children’s hospitals, are studying new chemotherapy combinations, which often include drugs such as topotecan, irinotecan, temozolomide, gemcitabine, docetaxel, and mithramycin (plicamycin).

Doctors are also trying to make the currently used drugs more effective by changing the way they are given. For example, they have found that giving the standard VAC/IE (VDC/IE) chemo regimen more often – that is, every 2 weeks instead of every 3 weeks – seems to lower the chance of localized Ewing tumors coming back, without increasing the risk of serious side effects. This is often called compressed chemotherapy.

Researchers are also studying high-dose chemotherapy with stem cell transplants in those with Ewing tumors that are unlikely to be cured with current treatments.

Targeted therapy

As noted in What Causes Ewing Tumors?, great progress is being made in understanding the changes in genes and chromosomes that cause Ewing tumors to form.

This knowledge has already been used to develop very sensitive lab tests to detect this cancer, and doctors are now studying how to best use these tests to guide the choice of treatment. It might also lead to new drugs that target these changes in Ewing tumor cells.

Some new drugs that target specific changes in Ewing tumor cells are already being tested. For example, TK216 is a drug that targets the main fusion protein in Ewing tumor cells that is thought to help these cells grow. Early studies of this drug in people with Ewing tumors are now under way.

Also being studied in clinical trials are drugs that target the insulin-like growth factor receptor-1 (IGF-1R), a protein on some cancer cells that causes them to grow. Early studies have found that drugs like this, such as ganitumab, can shrink some Ewing tumors and slow down the growth of others. So far, this benefit has been temporary in most cases. These drugs may work best when combined with other drugs.

Other newer drugs being studied for use against Ewing tumors include:

  • Drugs that affect a tumor’s ability to make new blood vessels, such as bevacizumab (Avastin)
  • Drugs that target the mTOR protein, such as temsirolimus (Torisel) and everolimus (Afinitor)
  • Drugs that target the PARP protein, such as olaparib (Lynparza) and nipararib (Zejula)
  • Drugs called HDAC inhibitors, such as vorinostat (Zolinza)

Immune therapy

A newer approach to treatment is to try to get the body’s own immune system to recognize and attack the tumor cells. Some newer types of immune therapies, such as checkpoint inhibitors, have shown a great deal of promise in treating other types of cancer, and some of these approaches are now being looked at for Ewing tumors. These treatments are still in the early stages of testing at this time.

The American Cancer Society medical and editorial content team

Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Anderson ME, Randall RL, Springfield DS, Gebhart MC. Chapter 92: Sarcomas of bone. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, Pa: Elsevier; 2014.

Gaspar N, Hawkins DS, Dirksen U, et al. Ewing sarcoma: Current management and future approaches through collaboration. J Clin Oncol. 2015;33(27):3036-3046.

Gebhart MC, DuBois S. Treatment of the Ewing sarcoma family of tumors. UpToDate. Accessed at www.uptodate.com/contents/treatment-of-the-ewing-sarcoma-family-of-tumors on March 12, 2018.

Last Medical Review: May 31, 2018 Last Revised: May 31, 2018

American Cancer Society medical information is copyrighted material. For reprint requests, please see our Content Usage Policy.