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Ørholt M, Abebe K, Rasmussen LE, et al.
J Am Acad Dermatol (2023)

Open AccessJC: December 2023

This nationwide population-based cohort study of 1,118 patients evaluated local recurrence and metastasis rates for atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). The study provided robust epidemiological data distinguishing the prognosis of these two related but distinct entities. PDS demonstrated significantly higher rates of recurrence and metastasis compared to AFX, supporting the clinical distinction between these tumors and informing follow-up protocols. The large sample size provides the most reliable outcome data to date for these uncommon tumors.

Take-Home Messages

  • PDS carries significantly higher recurrence and metastasis risk than AFX, confirming they require different management approaches.
  • AFX has an excellent prognosis with very low recurrence rates when completely excised, while PDS warrants closer surveillance.
  • These population-level data support standardized follow-up protocols: more intensive monitoring for PDS than AFX.

Topic

Rare & Special Tumors

DFSP, AFX, sebaceous carcinoma, EMPD, MAC, and other rare entities

Abstract

The prognosis of patients with atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) remains uncertain and no standardized follow-up programs have been established. To recommend a standardized follow-up program of patients with AFX and PDS based on nationwide long-term estimates of local recurrence and metastasis. All patients with AFX and PDS in Denmark between 2002 and 2022 were included. Danish National Registries were used to estimate the risks of local recurrence and metastasi

Literature review only. This summary is an editorial interpretation and may not reflect the complete findings of the original publication. Always refer to the full-text article for clinical decision-making.