Therapeutic Trial for Patients with Ewing Sarcoma and Desmoplastic Small Round Cell Tumors

Program Overview

This clinical trial is investigating treatments for two types of cancer: Ewing sarcoma and desmoplastic small round cell tumors. Participants with Ewing sarcoma are divided into two groups. Group A includes younger patients with tumors that haven't spread and are not in the pelvis. They will receive standard treatment. Group B includes older patients or those with tumors in the pelvis or that have spread. This group, along with those with desmoplastic tumors, will receive more intensive treatment due to a higher risk of the cancer returning. All participants will be monitored for 10 years after completing therapy.

Description

The main goal of this study is to see how well a combination of drugs works in treating high-risk Ewing sarcoma. These drugs include temsirolimus, temozolomide, and irinotecan. Researchers also want to see how long patients live without the cancer getting worse and how often the cancer returns after treatment.

Group A:

• Patients will receive chemotherapy every two weeks with drugs called vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. After a certain amount of doxorubicin, it will be stopped. Surgery or radiation will be done after six rounds of chemotherapy. Treatment lasts about 29 weeks.

Group B:

• Patients will start with two rounds of a special drug combination (temsirolimus, temozolomide, and irinotecan) over six weeks. Then, they will receive chemotherapy similar to Group A for several months. If the initial treatment works well, they will receive more of the special drug combination later. After chemotherapy, they will receive maintenance therapy with bevacizumab, sorafenib, and low-dose cyclophosphamide for additional support.

Eligibility Criteria

Locations