Trial Information

A Pilot Phase II Study for Children With Infantile Fibrosarcoma

OBJECTIVES:

Primary

- Determine the event-free and relapse-free survival of children with initially
unresectable congenital, infantile, or childhood fibrosarcoma treated with neoadjuvant
chemotherapy comprising vincristine, dactinomycin, and cyclophosphamide (VAC) before
definitive local control.

Secondary

- Determine the event-free and relapse-free survival of patients initially treated with
this regimen followed by observation after local control with positive microscopic
margins.

- Determine the event-free and relapse-free survival of patients initially treated with
this regimen followed by additional chemotherapy comprising etoposide and ifosfamide
after local control with gross positive margins.

- Determine the event-free and relapse-free survival of patients treated with surgery
alone.

OUTLINE: This is a pilot, multicenter study. Patients begin treatment according to lesion
resectability.

Patients with resectable lesions proceed to surgery.

- Surgery: Patients undergo resection of disease lesions. Patients with clear or
microscopically positive margins undergo observation only. Patients with grossly
positive margins undergo re-resection if feasible. Patients with grossly positive
margins after re-resection or for whom re-resection is not feasible receive
chemotherapy comprising vincristine, dactinomycin, and cyclophosphamide (VAC).

Patients with unresectable lesions receive VAC chemotherapy.

- VAC chemotherapy: Patients receive vincristine IV on days 1, 8, and 15 and dactinomycin
IV and cyclophosphamide IV over 1 hour on day 1. Treatment repeats every 21 days for up
to 6 courses in the absence of disease progression or unacceptable toxicity.

Patients with disease progression after 2-4 courses of VAC chemotherapy proceed to
chemotherapy comprising etoposide and ifosfamide (IE).

Patients with stable disease after 4 courses of VAC chemotherapy proceed to IE chemotherapy.

Patients with a partial response (PR) and unresectable lesions after 4 courses of VAC
chemotherapy receive 2 additional courses of VAC and are then re-evaluated. Patients proceed
to surgery if they continue to have a PR or achieve a complete response (CR) and lesions are
now resectable.

Patients with a CR or PR and resectable lesions after 4 courses of VAC chemotherapy proceed
to surgery.

Patients with stable disease, progressive disease, or a PR and unresectable lesions after 6
courses of VAC proceed to IE chemotherapy.

- IE chemotherapy: Patients receive etoposide IV over 1 hour and ifosfamide IV over 1
hour on days 1-5. Treatment repeats every 21 days for 4 courses in the absence of
disease progression or unacceptable toxicity.

Patients with a CR or PR and resectable lesions after 2-4 courses of IE chemotherapy proceed
to surgery.

All patients are followed every 3 months for 6 months, every 6 months for 1 year, and then
as clinically indicated.

PROJECTED ACCRUAL: A total of 60-70 patients will be accrued for this study within 8 years.