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

## TL;DR

This systematic review summarizes 33 cases of intra-oral aggressive fibromatosis, a rare soft tissue tumor, focusing on its clinical features, treatment, and recurrence rates.

## Contribution

The study provides a comprehensive synthesis of case reports and series on IOAF, highlighting treatment outcomes and recurrence patterns.

## Key findings

- IOAF predominantly affects males under 14 years old, with the mandible as the most common site.
- En bloc resection is the most effective treatment, with a 12.1% recurrence rate overall.
- Painless swelling is the most common clinical feature, and radiological findings often show ill-defined radiolucency.

## Abstract

Background/Objectives: 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. Methods: 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. Results: 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. Conclusions: 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.