Intra-Oral Aggressive Fibromatosis: A Systematic Review of Case Reports and Case Series

<b>Background/Objectives</b>: Intra-oral aggressive fibromatosis (IOAF) is a rare, locally invasive soft tissue tumor originating from fibroblasts. Despite its benign histological nature, IOAF exhibits a high recurrence rate and presents significant diagnostic and therapeutic challenges. This systematic review aims to synthesize the available literature on IOAF, focusing on clinical presentation, radiological and histological features, treatment modalities, and recurrence rates. <b>Methods</b>: A comprehensive systematic search was conducted, following PRISMA guidelines, across Pubmed, Cochrane, and ScienceDirect for case reports and series detailing IOAF published up to October 2025. This review was registered with PROSPERO (CRD42024586634). Data were extracted on demographics, clinical presentation, radiographic and histological findings, treatment strategies, and follow-up outcomes. Quality of included studies was assessed using the Joanna Briggs Institute (JBI) tools. <b>Results</b>: A total of 27 studies were included, encompassing 33 cases. IOAF predominantly affected males (54.5%) with a mean age of 13.15 years. The mandible was the most common site (69.7%). Painless swelling was the most frequent clinical feature (72.7%). Radiological findings were primarily ill-defined radiolucency in 54.5% of the cases. Histopathologically, spindle-shaped fibroblasts and collagen fibers were present in 90% of cases. En bloc resection was the most common treatment (60.6%), followed by excision (27.3%). Recurrence was observed in 12.1% of cases, primarily following conservative treatments. <b>Conclusions</b>: IOAF remains a challenging condition due to its locally aggressive nature and potential for recurrence. Surgical resection remains the primary treatment modality, with en bloc resection yielding the lowest recurrence rates. Further research into molecular pathogenesis and targeted therapies is needed to optimize treatment outcomes.