Extramammary Paget's disease in males: survival outcomes in 495 patients

Abstract

Background: Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy with poorly described outcomes. EMPD in men is primarily found in the perianal and penoscrotal areas. We assessed incidence and time trends of EMPD in men as well as extent and predictors of survival using the Surveillance, Epidemiology, and End Results (SEER) program.

Methods: The data from 18 SEER registries were queried for men diagnosed with EMPD from 1973 to 2009. Cases were categorized by primary skin location: perianal, penoscrotal, and other truncal. Additional data included age, race, presence of another cancer, tumor stage, and treatment (surgery, radiation, combination). Kaplan-Meier, univariate, and multivariate Cox regression analyses were conducted.

Results: Incidence rates of EMPD in men have been increasing with an annual percent change of +3.2 % (p < .0002) since 1978. Incidence of EMPD in blacks was nearly four times lower (p = .0003) and in Asians/Pacific islanders four times higher (p < .0001), relative to whites. Overall survival among 495 patients was 60.2 % at 120 months postdiagnosis. On multivariate analysis, significant factors negatively impacting survival were primary site in the perianal region compared to penoscrotal and truncal lesions (both p < .001), age older than 75 years (p < .001), and presence of distant versus localized disease (p = .018). Survival did not differ by race or presence of additional cancer.

Conclusions: Survival in men with EMPD is lower among those with distant disease and primary tumors located in the perianal region. The reasons for increasing EMPD incidence over time and for the racial disparities in disease occurrence require further study.