Jason P Sulkowski1, Mehul V Raval, Marybeth Browne. 1. Division of Pediatric Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, 700 Children's Drive, Columbus, OH 43205, USA.
Abstract
PURPOSE: The rarity of infantile fibrosarcoma (IF) has precluded comprehensive treatment evaluation. The purpose of this study was to better define the extent of surgical resection required and the role of chemotherapy. METHODS: Patients (0-2 years) with IF were evaluated from the National Cancer Data Base (1985-2007). Survival was estimated using the Kaplan-Meier method stratifying patients by margin status and treatment with or without chemotherapy. RESULTS: Of the 224 patients, 171 (76.3 %) were <1 year of age. Of the 64 (28.6 %) with positive margins, 36 (56.3 %) had microscopic disease, 12 (18.8 %) had macroscopic disease, and 16 (25 %) had unknown margin status; none were found to have metastases. Most were managed with surgical resection (171, 76.4 %). The proportion treated with both surgery and chemotherapy increased over time (18-40 %, p = 0.025). Disease-free survival was 90.6 %. No significant survival difference was noted in this retrospective, non-randomized cohort based on margin status, nodal involvement, tumor size, or treatment modality. CONCLUSIONS: The use of multimodal therapy has increased over time. There was a small increase in survival associated with negative margins and the use of multimodal therapy, however, neither result reached significance. Future studies investigating tumor biology and chemosensitivity will likely determine the optimal management of IF.
PURPOSE: The rarity of infantile fibrosarcoma (IF) has precluded comprehensive treatment evaluation. The purpose of this study was to better define the extent of surgical resection required and the role of chemotherapy. METHODS:Patients (0-2 years) with IF were evaluated from the National Cancer Data Base (1985-2007). Survival was estimated using the Kaplan-Meier method stratifying patients by margin status and treatment with or without chemotherapy. RESULTS: Of the 224 patients, 171 (76.3 %) were <1 year of age. Of the 64 (28.6 %) with positive margins, 36 (56.3 %) had microscopic disease, 12 (18.8 %) had macroscopic disease, and 16 (25 %) had unknown margin status; none were found to have metastases. Most were managed with surgical resection (171, 76.4 %). The proportion treated with both surgery and chemotherapy increased over time (18-40 %, p = 0.025). Disease-free survival was 90.6 %. No significant survival difference was noted in this retrospective, non-randomized cohort based on margin status, nodal involvement, tumor size, or treatment modality. CONCLUSIONS: The use of multimodal therapy has increased over time. There was a small increase in survival associated with negative margins and the use of multimodal therapy, however, neither result reached significance. Future studies investigating tumor biology and chemosensitivity will likely determine the optimal management of IF.
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