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Desmoid-type fibromatosis (DF) is a rare monoclonal fibroblastic proliferation that is characterized by locally infiltrative but rarely metastatic lesions.Tyrosine kinase and γ-secretase inhibitors are primarily used in the targeted therapy of DF.The use of these drugs,however,is mainly based on the recommendations of retrospective studies with small sample sizes.Previous studies that focused on the mechanism,efficacy,and safety of targeted therapy for DF were reviewed to provide references for clinical applications and research.The efficacy and safety of targeted therapy were compared with those of other systemic therapy options.Targeted therapy does not provide considerable advantages in efficacy and safety over other medical treatments and is usually applied after the failure of antihormonal therapies,nonsteroidal anti-inflammatory drugs,and chemotherapy.Further studies are required to explore the mechanism,indications,and appropriate drug dosage of the targeted therapy of DF.