Imatinib in Patients with Aggressive Fibromatosis and Pigmented Villonodular Synovitis: A Systematic Review

Introduction: We undertook a systematic review of the literature to determine the efficacy and safety of Imatinib in the restriction of activity and effective treatment of patients with aggressive fibromatosis (AF) and pigmented villonodular synovitis (PVNS) and their recurrence. Method: We searched studies in the PubMed and Cochrane collaborative library at all levels from ۲۰۱۰ to April ۲۰۱۹. Two independent reviewers evaluated the articles according to the predefined criteria and extracted the related data. Primary outcomes associated with tumor size shrinkage, relapse and overall survival along with secondary outcomes such as pain, quality of life, and side effects were investigated. Results: ۱۱۲ studies were evaluated out of which only ۶ studies that covered original studies and case reports were entered into this systematic review. A total of ۷۹subjects had participated in these six studies with an age range of ۲۲ to ۴۱ years old. In five studies, participants had a primary tumor and in one research they reported relapse. The recommended dose was ۴۰۰ mg/day, which was usually continued for ۴ to ۱۲ months. The tumor size shrinkage and low side effects of drugs have been reported. Conclusion: According to the results, no systemic treatment for PVNS and AF has been approved so far, but many studies have demonstrated the efficacy of Imatinib in the treatment of these diseases. However, further studies are required for optimal treatment and combined therapies.

Keywords:

Imatinib , aggressive fibromatosis , pigmented villonodular synovitis , Recurrence , Desmoids tumor