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<p>Abstract</p> <p>Background</p> <p>Surgery is still the standard treatment for aggressive fibromatosis (AF); however, local control remains a significant problem and the impact of R0 surgery on cumulative recurrence (CR) is objective of contradictory reports.</p> <p>Methods</p> <p>This is a single-institution study of 62 consecutive patients affected by extra-abdominal and intra-abdominal AF who received macroscopically radical surgery within a time period of 15 years.</p> <p>Results</p> <p>Definitive pathology examination confirmed an R0 situation in 49 patients and an R1 in 13 patients. Five-year CR for patients who underwent R0 vs R1 surgery was 7.1% vs 46.4% (<it>P</it> = 0.04) and for limbs vs other localizations 33.3% vs 9.9% (<it>P</it> = 0.02) respectively. In 17 patients who had intraoperative frozen section (IFS) margin evaluation R0 surgery was more common (17 of 17 vs 32 of 45, <it>P</it> = 0.01) and CR lower (five-year CR 0% vs 19.1%, respectively, <it>P</it> = 0.04). However, in multivariate analysis only limb localization showed a negative impact on CR (HR: 1.708, 95% CI 1.03 to 2.84, <it>P</it> = 0.04).</p> <p>Conclusions</p> <p>IFS evaluation could help the surgeon to achieve R0 surgery in AF. Non-surgical treatment, including watchful follow-up, could be indicated for patients with limb AF localization, because of their high risk of recurrence even after R0 surgery.</p>