BSV02 Malignancy screening rates and delayed malignancy diagnosis in patients with extramammary Paget disease: a nationwide cohort study using TriNetX

Abstract

While the clinical value of malignancy screening in patients with extramammary Paget disease (EMPD) is well established, screening rates, malignancy outcomes and delayed diagnosis among patients with EMPD remain poorly evaluated on a national scale in the USA. Using TriNetX, we identified 227 patients with an EMPD diagnosis across a national cohort during 2005–2025. We evaluated the time between EMPD diagnosis and first malignancy screening and the time to malignancy diagnosis in patients with and without malignancy screening. Relative risks, 95% confidence intervals and Kaplan–Meier survival curves were calculated with the TriNetX internal analytics package. In total, 61 patients (26.9%) received at least one malignancy screening test and 166 (73.1%) received no malignancy screening within 1 year of EMPD diagnosis. The proportion of patients receiving screening increased to 41.1% within 10 years following diagnosis, with a median time to first malignancy screening of 2237 days. Patients receiving at least one malignancy screening within 1 year of EMPD diagnosis were diagnosed with malignant neoplasms significantly earlier than their unscreened counterparts. In a subgroup analysis of adenocarcinomas and recurrent EMPD outcomes, the absence of any investigations within 1 year was associated with a greater time to malignancy diagnosis, with a median time of 669 vs. 232 days, respectively (P = 0.01; hazard ratio 1.65, 95% confidence interval 1.04–2.61). There was no significant difference in overall relative risk for malignancy over 10 years following EMPD diagnosis (relative risk 1.08, 95% confidence interval 0.98–1.19). Screened patients had similar malignancy risk to their unscreened counterparts. However, not receiving malignancy screening within 1 year was associated with a threefold greater time to diagnosis of a malignant neoplasm. This suggests delayed malignancy diagnosis for patients with EMPD who do not receive prompt screenings. While the obfuscation of small patient counts in TriNetX prevented site-specific malignancy associations, our analysis highlights the necessity for consistent, prompt screenings in clinical practice.