Trial of Chemotherapy Intensification Through Interval Compression in Ewing Sarcoma and Related Tumors, Phase III

Pts w/ Ewing sarcoma or primitive neuroectodermal tumor of the bone or soft tissue, excluding the CNS are eligible. Paraspinal tumors of extra-dural origin will be considered outside the CNS and are therefore eligible. Pts with Askin's tumor of the chest wall are also eligible. The tumor must be localized at the time of diagnosis. Pt w/ met. dz are not eligible. Pts w/ clinically or pathologically involved regional nodes are eligible; distant node involvement are not eligible. Tumors arising in the bony skull (extra-dural) are eligible; tumors arising in the intra-dural are not eligible. Tumor must have light microscopic appearance consistent w/ a Ewing family tumor of bone or soft tissue; additionally, there should be no IHC or ultrastructural evidence to exclude a Ewing family tumor (e.g. no evidence supporting a diagnosis of rhabdomyosarcoma). A panel of antibodies should be used of IHC characterization of any tumor suspected of being Ewing Sarcoma/PNET. In the absence of light microscopic, IHC or ultrastructural evidence, a diagnosis of RT-PCR or FISH evidence of an EWS rearrangement consistent w/ a Ewing family tumor may be used for diagnosis. Pts must not have received previous chemo or RT. Pts should only have had a bx of the primary tumor w/o an attempt at complete or partial excision; however, pts will still be eligible if excision was attempted or accomplished. Pts must have CrCl or isotope glomerular filtration rate >= 75 ml/min/1.73m2; bili <= 1.5 mg/dL total; adequate cardiac function (defined as shortening fraction >= 28% by ECG or Ejection fraction >= 55% by radionuclide angiogram). Pts of childbearing potential must be willing to use effective contraception. Pregnant or lactating pts are not eligible. Pts must <=50 years of age at diagnosis