Purpose of study:
In this study, researchers want to find out if we can improve the treatment for your disease by adding another drug called topotecan to the 5 drugs used in standard treatment.
We chose the drug topotecan because the combination of topotecan and cyclophosphamide has been found to be useful in treating some subjects with Ewing sarcoma when it has come back after treatment (recurrence). The combination of vincristine/topotecan/cyclophosphamide (VTC) has been well-tolerated in people with other types of sarcoma. A recent COG study added VTC to standard therapy for localized Ewing sarcoma and the early results indicate that VTC can be used in this way.
The overall goal of this study is:
To find if adding the drug combination VTC to the standard five-drug chemotherapy for Ewing sarcoma will get rid of the cancer better than the standard five-drug chemotherapy by itself. ?
Who can participate:
Patients with newly diagnosed, biopsy confirmed, extracranial, non-metastatic Ewing sarcoma or PNET of bone or soft tissue are eligible for this study.
No prior chemotherapy or radiation therapy is allowed. Patients should only have had a biopsy of the primary tumor without an attempt at complete or partial resection. Patients will still be eligible if unplanned excision was attempted or accomplished as long as adequate imaging was obtained prior to surgery.
Patient must be < 30 years of age at the time of study enrollment.
Additional screening for eligibility will be required.
What will happen during the study:
Subjects (people participating in the study) will receive 1 of 2 different treatment plans. The treatment plan they receive is decided by a process called randomization. Randomization means that the treatment is assigned based on chance. It is a lot like flipping a coin, except that it is done by computer to make sure that there are about the same number of people on each treatment plan of the study. The randomization process is described in the COG Family Handbook for Children with Cancer. You will be randomized to get either standard chemotherapy (Regimen A) or experimental chemotherapy (Regimen B). The randomization takes place at the time you are enrolled on the study. You have an equal chance of getting either one of the treatments.
The treatment plan involves chemotherapy along with surgery and/or radiation therapy. The treatment on this clinical trial may take about 9 months. The treatment is divided into 3 stages: Induction, Local control and Consolidation therapy.
Each cycle of chemotherapy on this study will last 14 days. Induction will be 6 cycles of chemotherapy, and will last around 12 weeks. Local Control will begin upon recovery from Induction and may be surgery, radiation therapy, or both (as described below). That decision will be made by your doctors and will depend on the location and size of the tumor. Consolidation includes 11 cycles (22 weeks) of chemotherapy and may start during the period of Local Control. This means that sometimes radiation therapy and some chemotherapy will be given in the same weeks.
If you are to have surgery only, this will happen after you recover from Induction therapy.
The exact timing of chemotherapy and local control will depend on how well you recover from the drugs given during each cycle. You will also need time to heal from any surgery, and from the effects of radiation therapy. ?
Subjects in this clinical trial are expected to receive treatment on this study for about 9 months. After treatment, subjects will have follow-up examinations and medical tests. We will continue to collect some medical information about how you are doing for 10 years from the time you start the study.
Who to contact:
PI: Mark Ranalli, MD