BACKGROUND: The causes of death in extramammary Paget's disease (EMPD) patients are currently unclear. The authors aimed to examine mortality patterns among current survivors of EMPD. METHODS: We conducted a retrospective cohort analysis utilizing data from the Surveillance, Epidemiology, and End Results (SEER) database spanning from 2000 to 2019, focusing on patients diagnosed with EMPD. Standardized mortality ratios (SMRs) were employed to assess mortality rates in EMPD patients compared to the general population. RESULTS: Our study comprised 1,002 patients diagnosed with EMPD, among whom 437 (43.6%) patients died during the follow-up period. In patients staged as localized, all malignant tumors accounted for 29.71% (n=101) of all deaths. Except for the 5-<
10 years SMR being higher than the general population, no substantial difference was observed in SMR between EMPD and the general population for all causes of death. Among patients with regional/distant EMPD, 55 (56.70%) individuals died from all malignant tumors, while 42 (43.30%) individuals died from non-cancer causes. Throughout the follow-up period, the all-cause mortality rate was higher than that of the general population [SMR, 1.91
95% confidence interval (CI): 1.55-2.33]. Cardiovascular and cerebrovascular diseases were the most common non-cancer causes of death in the whole cohort. Compared to patients aged 60-70 years and those aged 70 years and above, patients under 60 years had a lower SMR for all-cause mortality. CONCLUSIONS: Non-cancer related fatalities represent over 70% of cases in localized stage and 40% in regional/distant stage. Although the risk of all malignant tumors death is higher than that of the standard population, the overall risk of death for patients with localized stage is not increased. These data provide most up-to-date and comprehensive analysis of mortality causes among EMPD patients.