The incidence of atypical fibroxanthoma and pleomorphic dermal sarcoma in Denmark from 2002 to 2022

Background

The purpose of this study was to assess trends in the incidence rates and overall survival of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) in the Danish population from 2002 to 2022.

Methods

We included all patients diagnosed with AFX or PDS in the Danish National Pathology Register (DNPR) during the study period. We computed the age-adjusted incidence rate (AAIR) per 100,000 and the average annual percent change (AAPC) and developed an age-period-cohort (APC) model of incidence.

Results

We included a total of 1118 patients, 78.3 % of whom were men. The median age upon diagnosis for AFX and PDS was 77.6 and 78.4 years, respectively. 84.5 % of the 1118 patients received an AFX diagnosis, while 15.5 % received a PDS diagnosis. The AAIR rose from 0.2 per 100,000 individuals in 2002 to 0.5 per 100,000 by 2022, with a peak increase of 0.75 per 100,000 in 2014. The AAPC for both tumors was 5.3 (95 % CI 2.9 %–7.7 %). We found a significant difference in overall survival between AFX and PDS, with PDS having worse overall survival than AFX.

Conclusion

This study, which included the largest non-selected national population to date, found a significant increase in the incidence of AFX and PDS between 2002 and 2015, followed by a decrease.