Atypical Fibroxanthoma: Trends and Insights From a Single Tertiary Care Institution Over a Ten-Year Span

Atypical fibroxanthoma (AFX) can be difficult to distinguish from other fibrohistiocytic tumors. Accurate diagnosis and management require clear understanding of histopathologic and clinical features, including tumor behavior and outcomes.

To evaluate the histopathologic and clinical characteristics of AFX and pleomorphic dermal sarcoma (PDS)/undifferentiated pleomorphic sarcoma (UPS) cases treated at a single tertiary care center over a 10-year period.

A retrospective chart review was performed for patients diagnosed with AFX and PDS/UPS between 2013 and 2023. Patient demographics, tumor features, treatment modalities, and outcomes were recorded. Statistical analysis utilized the Fisher exact test and Wilcoxon rank-sum test.

The study identified 108 patients with 113 AFX tumors and 5 PDS cases, including 2 cases of AFX progressing to PDS. The mean age at diagnosis was 74 years. Two patients were immunosuppressed. 69.4% of patients had a history of malignancy. The scalp was the most common tumor site (38.9%). Among 111 tumors treated with Mohs micrographic surgery, 43.2% cleared in 1 stage. Four patients experienced local recurrence; one developed metastasis after progression to PDS.

AFX carries a risk of recurrence and progression. Accurate diagnosis, intraoperative histologic evaluation, and close follow-up are essential.