How is infantile fibrosarcoma (IFS) treated?

Updated: Dec 03, 2018
  • Author: Ian D Dickey, MD, FRCSC, LMCC; Chief Editor: Omohodion (Odion) Binitie, MD  more...
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Sulkowski et al conducted a retrospective, nonrandomized study aimed at defining the extent of surgical resection needed in the treatment of infantile fibrosarcoma (IFS), as well as the role of chemotherapy in management. [13] The study cohort consisted of 224 patients aged 0-2 years. Of the 64 patients (28.6%) with positive margins, 36 (56.3%) had microscopic disease, and 12 (18.8%) had macroscopic disease; margin status was unknown for 16 (25%). None of the patients had metastases. In all, 171 (76.4%) were treated with surgical resection.

The disease-free survival rate was 90.6%. [13] No significant survival difference was noted with regard to margin status, nodal involvement, tumor size, or treatment modality. The use of multimodal therapy (surgery in conjunction with chemotherapy) increased over time. A small increase in survival was associated with negative margins and multimodal therapy, but neither result was statistically significant. The authors suggested that future studies investigating tumor biology and chemosensitivity may determine optimal management of IFS.

The European Paediatric Soft Tissue Sarcoma Study Group evaluated a conservative therapeutic strategy in 50 infants (median age, 1.4 months) with localized IFS. [14] Initial surgery was suggested only if it could be done without mutilation. No further therapy was administered to those with initial complete (group I/R0; n=11) or microscopic incomplete (group II/R1; n=8) resection; vincristine-actinomycin (VA) chemotherapy was administered to those with initial inoperable tumors (group III/R2; n=31), with delayed conservative surgery planned after tumor reduction.

After delivery of VA chemotherapy to 25 children with group III/R2 and one with group II/R1 disease, there was a 68% rate of response to VA; only three children required mutilating surgery. [14] The 3-year event-free survival was 84% and the overall survival 94.0% at a median follow-up of 4.7 years (range, 1.9-9.0 years). Alkylating or anthracycline based chemotherapy was avoided in 71.0% of patients needing chemotherapy.


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