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High risk of proximal and local neoplasms in 2,206 patients with anogenital extramammary Paget disease

Diseases of the Colon and Rectum Oct 16, 2019

Lee GC, Kunitake H, Stafford C, et al. - Patients with extramammary Paget disease were examined for the risk of second primary neoplasms. Researchers conducted a retrospective analysis of the Surveillance, Epidemiology, and End Results Registry (1973–2014) identifying 108 patients with anal Paget disease, 421 patients with male genital (scrotum or penis) Paget, and 1,677 patients with female genital (vagina or vulva) Paget. During the median follow-up time of 5.9 years. Patients with anal Paget disease had 18.5% risk of developing colorectal adenocarcinoma; male patients with genital Paget had 9.7% risk of proximal genitourinary malignancy, 0.4% risk for scrotal or testicular adenocarcinoma, and 1.7% risk for penile or scrotal squamous carcinoma. Female patients with genital Paget exhibited 3.0% risk of proximal genitourinary malignancy, 1.4% risk for vaginal or vulvar adenocarcinoma, and 1.0% risk for vaginal or vulvar squamous neoplasm. Five-year overall survival was 59.7%, 73.5%, and 80.7% in patients with anal, male genital, and female genital Paget, this indicates worse survival among Patients with anal Paget relative to those with purely genital Paget.
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