High Risk of Proximal and Local Neoplasms in 2206 Patients With Anogenital Extramammary Paget’s Disease

BACKGROUND: Extramammary Paget’s disease is an uncommon intraepidermal adenocarcinoma with poorly defined clinical implications. OBJECTIVE: The purpose of this research was to estimate the risk of second primary neoplasms in patients with extramammary Paget’s disease. DESIGN: This was a retrospective analysis of the Surveillance, Epidemiology, and End Results Registry (1973–2014). SETTINGS: The study included population-based cancer registries from the United States. PATIENTS: Patients who were diagnosed with anogenital Paget’s disease were included. MAIN OUTCOME MEASURES: Risk of second primary development was measured. RESULTS: We identified 108 patients with anal Paget’s disease, 421 patients with male genital (scrotum or penis) Paget’s, and 1677 patients with female genital (vagina or vulva) Paget’s. Median follow-up time was 5.9 years. The risk of developing colorectal adenocarcinoma was 18.5% for patients with anal Paget’s disease. Eighty percent of colorectal adenocarcinoma diagnoses were synchronous (within 2 mo) to anal Paget’s diagnoses, whereas metachronous tumors occurred at a median time of 2.4 years. Of patients with anal Paget’s disease, 8.3% developed an anal adenocarcinoma or nonsmall cell cancer. In male patients with genital Paget’s, the risk of proximal genitourinary malignancy was 9.7%, scrotal or testicular adenocarcinoma was 0.4%, and penile or scrotal squamous carcinoma was 1.7%. In female patients with genital...
Source: Diseases of the Colon and Rectum - Category: Gastroenterology Tags: Original Contributions: Perianal Neoplasia Source Type: research