Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Retrospective Review and Survival Analysis at a Single Academic Institution.

Abstract

Background: Prognostication of atypical spindle cell neoplasms, including atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS), is challenging; outcomes vary widely, and further identification of prognostic features is crucial.

Objective: To evaluate prognostic factors that may portend worse outcomes in patients with AFX and PDS.

Materials and methods: A retrospective chart review of patients with AFX and PDS was conducted. Data were analyzed using Pearson chi square and T-test. Survival was analyzed using the Kaplan-Meier estimate. A stepwise cox proportional hazards model was created to identify variables associated with increased risk of poor outcomes.

Results: Thirty-four patients with AFX and 10 patients with PDS were identified. PDS had a 60% overall mortality rate (vs 26.5% in AFX), a larger preoperative size of 8.9 cm2 (vs 2.4 cm2) (p < .0001), and a 50% recurrence rate (vs 5.9%) (p = .0039). Female gender (HR 22.18 95% CI 2.54-194.13; p = .001), immunosuppression (HR 5.02 95% CI 1.32-19.12; p = .0044), and perineural invasion (PNI) (HR 78.46 3.97-1,549.56; p = .0091) were associated with increased risk of poor outcomes.

Conclusion: Few large studies have examined clinical features that portend worse outcomes. The authors identify female gender, immunosuppression, and PNI as variables associated with increased risk of recurrence or death.