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Extramammary Paget's disease in males: survival outcomes in 495 patients
Lindsey A Herrel1, Aaron D Weiss, Michael Goodman
1Department of Urology, Emory University, Atlanta, GA, USA.
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.
Insights
Extramammary Paget's disease (EMPD) survival in men is lower for perianal tumors and older age. Incidence of this rare cancer is increasing, with racial disparities observed.
Area of Science:
- Oncology
- Epidemiology
- Dermatology
Background:
- Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy with limited outcome data.
- In men, EMPD predominantly affects the perianal and penoscrotal regions.
- Understanding incidence, trends, and survival predictors is crucial for this patient population.
Purpose of the Study:
- To assess the incidence and time trends of EMPD in men.
- To determine the extent and predictors of survival for male EMPD patients.
- To analyze demographic and clinical factors influencing EMPD outcomes.
Main Methods:
- Utilized data from 18 Surveillance, Epidemiology, and End Results (SEER) registries (1973-2009).
- Categorized cases by primary skin location (perianal, penoscrotal, other truncal).
- Employed Kaplan-Meier, univariate, and multivariate Cox regression analyses.
Main Results:
- EMPD incidence in men shows a significant annual increase of +3.2% since 1978.
- Incidence was lower in Black men and higher in Asian/Pacific Islander men compared to White men.
- Negative survival predictors included perianal primary site, age >75 years, and distant disease stage.
Conclusions:
- Survival outcomes for men with EMPD are poorer with distant disease and perianal primary tumors.
- The rising incidence and observed racial disparities in EMPD require further investigation.
- Identifying specific reasons for these trends and disparities is essential for future research and clinical management.
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