Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma

DISEASE CHARACTERISTICS:

• Newly diagnosed extracranial, non-metastatic Ewing sarcoma or primitive neuroectodermal tumors of bone or soft tissue

  • For the purpose of this study, any of the following are considered localized disease:

    • Chest wall tumors with ipsilateral pleural effusions
    • Ipsilateral positive pleural fluid cytology
    • Ipsilateral pleural-based secondary tumor nodules
  • Regional node involvement, based on clinical suspicion confirmed by pathologic documentation, are considered to be non-metastatic disease
  • Tumors arising in the bony skull (extra-dural) are considered to be extracranial

• No evidence of metastatic disease, including the following:

  • Lesions that are discontinuous from the primary tumor, are not regional lymph nodes, and do not share a body cavity with the primary tumor
  • Contralateral pleural effusion and contralateral pleural nodules
  • Distant lymph node involvement

  • Pulmonary nodules that meet the following criteria:

    • Solitary nodule > 0.5 cm or multiple nodules of > 0.3 cm unless biopsied and negative for Ewing sarcoma
    • Biopsies of solitary nodule < 0.5 cm or multiple nodules < 3.0 cm (are not required but if performed) positive for metastatic disease
• No tumors arising in the intra-dural soft tissue

PATIENT CHARACTERISTICS:

• Creatinine clearance or radioisotope GFR ≥ 70 mL/min OR serum creatinine based on age and/or gender as follows:

  • 0.4 mg/dL (1 month to < 6 months of age)
  • 0.5 mg/dL (6 months to < 1 year of age)
  • 0.6 mg/dL (1 year to < 2 years of age)
  • 0.8 mg/dL (2 years to < 6 years of age)
  • 1.0 mg/dL (6 years to < 10 years of age)
  • 1.2 mg/dL (10 years to < 13 years of age)
  • 1.5 mg/dL (male) or 1.4 mg/dL (female) (13 years to < 16 years of age)
  • 1.7 mg/dL (male) or 1.4 mg/dL (female) (≥ 16 years of age)
• Total bilirubin < 1.5 times upper limit of normal (ULN)
• AST or ALT < 2.5 times ULN
• Shortening fraction of ≥ 27% by echocardiogram OR ejection fraction ≥ 50% by radionuclide angiogram
• Not pregnant or nursing
• Negative pregnancy test
• Fertile patients must use effective contraception for the duration of study treatment

PRIOR CONCURRENT THERAPY:

• No prior chemotherapy or radiotherapy

• Prior biopsy of the primary tumor without an attempt at complete or partial resection allowed

  • Patients are still allowed if unplanned excision was attempted or accomplished as long as adequate imaging was obtained prior to surgery
• No other concurrent chemotherapy or immunomodulating agents (including steroids unless used as an antiemetic)
• No concurrent sargramostim (GM-CSF)