Treatments for Ewing sarcoma

Treatment for Ewing sarcoma consists of neoadjuvant chemotherapy, followed by local treatment (surgery and/or radiotherapy) and then adjuvant chemotherapy.

Pediatric, adolescent, and young adult patients being treated for Ewing sarcoma receive care at Institut Curie's SIREDO unit, an expert center for care, innovation, and research in childhood and adolescent cancer.

Institut Curie also calls on specialists in adult sarcomas, who offer their expertise as part of a collaboration with pediatric oncologists to tailor treatments to adolescents and young adults.

Chemotherapy for Ewing sarcoma

The primary purpose of neoadjuvant chemotherapy is to stop the progression of the disease and facilitate surgery by reducing the tumor’s size.

The treatment alternates every two weeks between vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide. Maintaining the dose intensity is essential for optimal control of the disease. Imaging is used to assess the tumor after four and nine treatments.

After local treatment, patients receive so-called adjuvant chemotherapy, which is often virtually identical to the neoadjuvant regimen. In some cases, high-dose chemotherapy with hematopoietic stem cell rescue may be given.

For patients with metastatic disease, maintenance therapy combining oral and intravenous chemotherapy can be administered in an outpatient setting.

The next European treatment protocol for treatment of Ewing sarcoma patients, InterEwing, is expected to open at Institut Curie in 2025.


 

Surgery for Ewing sarcoma

When surgery is indicated, it is performed in one of the pediatric orthopedic departments within the Paris public hospital system (Necker, Trousseau, Robert-Debré) or at Cochin Hospital for adolescents and young adults.

The goal is a complete macroscopic and microscopic resection. 

The surgical specimen is then analyzed histologically to evaluate the resection quality and chemotherapy response; these will determine the need for adjuvant treatment, particularly radiotherapy.

Radiotherapy for Ewing sarcoma

Ewing sarcoma is radiosensitive, so radiotherapy plays a central role in its treatment.
It may be adjuvant (after surgery) or exclusive (as the only local treatment). 
Treatment modalities are defined by the radiation oncologists. Two main techniques are used: photon therapy and proton therapy. The latter, provided at the Proton Therapy Center in Orsay, is preferred when the tumor is near sensitive organs such as the spinal cord, heart, or brain. Radiotherapy can be performed under general anesthesia at Institut Curie, particularly for very young children.
 

Find out more about the Proton Therapy Center in Orsay - Institut Curie

Targeted therapies for Ewing sarcoma

By blocking specific mechanisms involved in tumor growth and proliferation, targeted therapies offer a new approach to treating Ewing sarcoma. Tyrosine Kinase Inhibitors (TKIs) are the main class of drugs used, sometimes as a first-line treatment when the cancer is well advanced, or otherwise as maintenance treatment or in the event of a relapse.