Extra-osseous Ewing's sarcoma metastatic Trials in Disease

Study of drug combination therapy for patients with recurrent and primary refractory Ewing Sarcoma

This trial is designed for patients whose Ewing sarcoma has either returned after treatment or did not respond to the first treatment. The study compares several different combinations of chemotherapy drugs to determine which works best.

Main inclusion criteria:

• Patients must be at least 2 years old
• The diagnosis of Ewing sarcoma or Ewing-like sarcoma must be confirmed through tissue examination
• Evidence that the disease has progressed during or after completing treatment
• Patients must be medically fit enough to receive the trial treatment
• Adequate kidney function is required, with a measurement called GFR of 60 or higher
• Imaging tests must be completed within 4 weeks before starting the trial
• For female patients who can become pregnant, a negative pregnancy test is required
• Patients must be willing to use effective birth control during treatment and for 12 months afterward
• For certain treatment options, additional requirements include: adequate liver function, heart function with an ejection fraction of at least 50%, controlled blood pressure, and acceptable protein levels in urine

Main exclusion criteria:

• Patients who have not been diagnosed with Ewing sarcoma
• Patients younger than 6 years old or older than 65 years of age
• Patients whose cancer has not returned or who have not shown resistance to initial treatment
• Patients who cannot undergo systemic therapy that affects the whole body
• Patients who cannot complete the required imaging tests
• Patients with severe medical conditions that would make participation unsafe
• Patients currently participating in other clinical trials that could interfere with this study
• Patients who are pregnant or breastfeeding
• Patients with known allergies to the study medications
• Patients who cannot comply with study requirements or follow-up visits

Focus and goal of the trial:

The main goal of this study is to find out which combination of chemotherapy drugs works best for treating Ewing sarcoma that has returned or did not respond to initial treatment. The trial tests multiple chemotherapy medications, including gemcitabine, docetaxel, irinotecan, temozolomide, topotecan, carboplatin, cyclophosphamide, ifosfamide, etoposide, and lenvatinib. These medications are given in different combinations, either through an intravenous infusion into a vein or as oral capsules taken by mouth.

Treatment cycles typically last several weeks, and some medications are given daily while others are given on specific days. The total treatment can continue for up to 104 weeks, depending on how well it works for each patient.

Throughout the study, doctors monitor the size of tumors using imaging scans to check if the treatment is working. They also track any side effects and measure how long patients live without their disease getting worse. The study also evaluates patients' quality of life during treatment. Regular monitoring includes checking blood pressure, liver function, kidney function, and heart function for specific treatment groups.

Investigational drugs:

The trial involves several chemotherapy medications: gemcitabine, docetaxel, irinotecan, temozolomide, topotecan, carboplatin, cyclophosphamide, ifosfamide, etoposide, and lenvatinib. These drugs are used in different combinations to find the most effective treatment approach.