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Background  Aggressive fibromatosis (AF) is a rare fibroblastic proliferative disease with a locally aggressive behavior and no distant metastasis, characterized by driver mutations in  CTNNB1  or the  APC  gene. When progressive and/or symptomatic AF is not amenable to local management, a variety of medical treatments may be efficient, including imatinib mesylate. The phase II “Desminib trial” included 40 patients with AF to evaluate the toxicity and efficacy of imatinib resulting in a 65% tumor control rate at 1 year. We investigated a potential predictive value of  KIT  exon 10 M541L variant ( KIT  L541 ) on this prospective series.    Methods  DNA was extracted in sufficient quantity from 33 patients included in the Desminib trial. The detection of  KIT  L541  was performed by Competitive Allele-Specific Taqman® PCR technology. Chi-2 analyses were performed to search for a correlation between KIT status and tumor response. Progression free (PFS) and overall survival (OS) were compared by log-rank test after Kaplan-Meier analysis.    Results  In 6 out of 33 cases (18%), the technique failed to determine the mutational status; 5 patients (19%) harboured  KIT  L541  and 22 patients (81%) were classified as  KIT  wild type. Compared with total cohort,  KIT  L541  frequency did not distinguish between different clinical characteristics. In the  KIT  L541  and the  KIT  WT  subgroups, the tumor control rate at 1 year was 100% and 68%, respectively (p = 0.316). The median PFS of patients harboring  KIT  L541  or not is 29.9 and 24.5 months, respectively (p = 0.616), and the median OS is not reached, in any of the groups.    Conclusion  Our results do not support a predictive effect of  KIT  L541  on the efficacy of imatinib for patients with AF.

bmc cancer

Impact of KIT exon 10 M541L allelic variant on the response to imatinib in aggressive fibromatosis: analysis of the desminib series by competitive allele specific Taqman PCR technology