Vincristine Sulfate, Topotecan Hydrochloride, and Cyclophosphamide With or Without Bevacizumab in Treating Young Patients With Refractory or First Recurrent Extracranial Ewing Sarcoma

PRIMARY OBJECTIVES: I. To determine the feasibility of administering bevacizumab in combination with vincristine (vincristine sulfate), topotecan hydrochloride, and cyclophosphamide (VTC) to younger patients with refractory or first recurrent Ewing sarcoma. II. To compare the progression-free survival of patients treated with VTC with bevacizumab vs VTC without bevacizumab. SECONDARY OBJECTIVES: I. To estimate the response rate to 2 cycles of VTC compared to 2 cycles of VTC/bevacizumab. II. To evaluate biological markers as related to prognosis and specifically related to angiogenesis by encouraging concurrent enrollment on the Ewing sarcoma banking studies (COG-AEWS02B1 and/or COG-AEWS07B1) and ancillary correlative endothelial cell, surrogate marker, and angiogenic gene studies. OUTLINE: This is a single therapy feasibility study followed by a randomized controlled portion. Patients are stratified according to time to disease recurrence (\< 2 years vs \>= 2 years). ARM I (Feasibility assessment of VTCB): Patients receive bevacizumab intravenously (IV) over 30-90 minutes on day 1, vincristine sulfate IV on days 1, 8, and 15, and topotecan hydrochloride IV over 30 minutes and cyclophosphamide IV over 60 minutes on days 1-5. Treatment repeats every 21 days (except during weeks 14, 15 \[course 5\], 17, 18 \[course 6\], 26, 27 \[course 9\], 29, and 30 \[course 10\] when no chemotherapy is given) for up to 12 courses in the absence of disease progression or unacceptable toxicity. ARM II (VTCB): Patients receive bevacizumab, vincristine sulfate, topotecan hydrochloride, and cyclophosphamide as in Arm I. ARM III (VTC): Patients receive vincristine sulfate, topotecan hydrochloride, and cyclophosphamide as in arm I. After completion of study therapy, patients are followed periodically.