Surgery, observation, and/or chemotherapy

Complete resection is curative in most patients with infantile fibrosarcoma. However, the large size of the lesion frequently makes resection without major functional consequences impossible. For instance, tumors of the extremities often require amputation for complete excision.

The European pediatric group has reported that observation may also be an option in patients with microscopic residual disease after surgery.[88] Twelve patients with microscopic residual disease received no further therapy and two patients experienced disease relapse. One patient obtained a complete remission after chemotherapy. Postoperative chemotherapy was administered to patients with higher group disease and those who progressed. In a subsequent study, only one of seven patients with microscopic residual disease progressed during observation. That patient achieved complete remission with chemotherapy.[89][Level of evidence C1]

Preoperative chemotherapy has made a more conservative surgical approach possible. Agents active in this setting include vincristine, dactinomycin, cyclophosphamide, and ifosfamide.[90,91]; [89,92,93][Level of evidence C1] Three older studies of patients with infantile fibrosarcoma suggested that an alkylator-free regimen was effective and used as the first treatment choice in patients with macroscopic disease.[88,89,94] However, newer results of studies using NTRK inhibitors have suggested that kinase inhibitors are an appropriate initial therapy.