Objective  To evaluate the efficacy and safety of anlotinib in patients with extremity desmoid-type fibromatosis (DF).

  Methods  Clinical and follow-up data of 26 patients with extremity DF who received anlotinib between January 2019 and April 2020 in West China Hospital were retrospectively analyzed .

  Results  All the 32 patients were followed up regularly with a median follow-up of 14 (11.75-16) months. At last follow-up, none of the patients achieved a complete response (CR), but ten patients (38.5%) achieved a partial response (PR) and twelve patients (46.1%) achieved stable disease (SD), four patients (15.4%) developed progressive disease (PD) and the 6-, 12-month progression-free survival (PFS) rates were 92.3%, 84.6% respectively. The disease control rate (DCR) was 84.6% (22/26) and the objective response rate (ORR) was 38.5% (10/26). Meanwhile, a reduction in tumor size was achieved in eighteen patients (69.2%), a decrease in T2-weighted signal intensity on magnetic resonance imaging (MRIT2) was observed in 22 patients (84.6%), a subjective diminishment in local pain was described in 25 patients (96.2%), and an improvement in the range of motion of adjacent joints was examined in nineteen patients (73.1%) after anlotinib treatment. Additionally, anlotinib-induced toxicities were tolerated for all patients with extremity DF.

  Conclusions  Anlotinib was effective against extremity DF and significantly slowed the disease progression to finally improve the quality of patients’ life with an acceptable drug-related toxicity.