Clinical Outcomes and Prognosis in Atypical Fibroxanthoma: A Systematic Review and Meta-Analysis.

Abstract

Atypical fibroxanthoma (AFX) is a rare dermal spindle-cell tumor characterized by a generally benign clinical course. Despite recent advances, there are still challenges regarding its accurate diagnosis, prognostic indicators, and optimal management strategies. This systematic review and meta-analysis aims to evaluate recurrence rates, identify prognostic factors, and provide evidence-based insights for managing AFX. We systematically searched the PubMed, Embase, Web of Science, and Cochrane databases following PRISMA guidelines (PROSPERO: CRD42024507089). Observational studies published from 2002 to 2024 that confirmed AFX through histopathology and immunohistochemistry were included. Recurrence rates and prognostic factors were analyzed using a random-effects model with inverse-variance weighting. Sensitivity analysis assessed heterogeneity, while publication bias was evaluated via funnel plots and Egger's test. Eight studies involving 1366 patients were analyzed. Patients were predominantly elderly males (78.9%), with an average age of 76.9 years, and lesions mostly in the head and neck region (89%), measuring about 14.9 mm in diameter. The pooled local recurrence rate was 4.73% [95% confidence interval (CI): 2.24-9.73%; I² = 62.1%], while the metastasis rate was 0.59%, and disease-specific mortality was 0%. Strict adherence to standardized diagnostic criteria results in low recurrence rates in AFX, reinforcing its predominantly benign clinical behavior. Therefore, we highlight the importance of maintaining strict diagnostic standards for AFX to ensure accurate patient prognosis.