Trial Information

Actinomycin D and Vincristine With or Without Radiation Therapy, for Newly Diagnosed Patients With Low-Risk Rhabdomyosarcoma or Undifferentiated Sarcoma: IRS-V Protocol

OBJECTIVES:

- Determine the failure-free survival (FFS) rate in patients with newly diagnosed
low-risk rhabdomyosarcoma of embryonal or botryoid subtype meeting criteria for group I
after treatment with dactinomycin and vincristine with or without radiotherapy.

- Determine the FFS rate in these patients meeting criteria for group II after treatment
with dactinomycin, vincristine, and cyclophosphamide with or without radiotherapy.

- Determine the FFS rate in patients with ectomesenchymomas containing
rhabdomyosarcomatous elements (embryonal histiotype) who receive one of the above
treatments.

- Determine new molecular markers specific to embryonal and botryoid tumor histologies
which are of diagnostic and prognostic significance in patients treated with these
regimens.

OUTLINE: Patients are assigned to 1 of 2 groups, depending on histology and site of disease.

- Group I (favorable tumor site, negative lymph nodes, stage 1, clinical group I, IIA, or
III (orbit only), node negative [N0] OR unfavorable tumor site, negative or unknown
lymph nodes, stage 2, clinical group I): Patients receive vincristine IV over 1 minute
weekly for 8 weeks and dactinomycin IV over 1 minute once every 3 weeks for 4 doses.
Treatment repeats every 12 weeks for 4 courses. Radiotherapy is administered to
patients with clinical group II or III disease on weeks 3-8.

- Group II (favorable tumor site, positive lymph nodes, stage 1, clinical group III
(orbit only), node positive [N1] OR favorable tumor site except orbit, any lymph nodes,
stage 1, clinical group III OR unfavorable tumor site, stage 2, clinical group II OR
unfavorable tumor site, stage 3, clinical group I or II): Patients receive vincristine
and dactinomycin as in group I. Patients also receive cyclophosphamide IV over 30-60
minutes and filgrastim (G-CSF) or sargramostim (GM-CSF) subcutaneously once daily
beginning 24 hours after completion of chemotherapy and continuing for 10 days or until
blood counts recover. Radiotherapy is administered on weeks 3-8, 12-17, or 28-33, if
clinically indicated as in group I.

Patients are followed every 3-4 months for 3 years (4 years after diagnosis), every 6 months
for 1 year, and then annually thereafter.

PROJECTED ACCRUAL: A total of 254 patients for group I will be accrued for this study within
6 years. Approximately 12 patients per year will be accrued for group II.