Management and outcome of perianal Paget's disease: a 6-decade institutional experience.

BACKGROUND: Perianal Paget's disease (intraepithelial adenocarcinoma) is rare and sometimes difficult to diagnose because symptoms are nonspecific. It is often noninvasive but frequently recurs locally. Invasive disease can metastasize to distant sites.

OBJECTIVE: The purpose of this work was to review the diagnosis, management, and outcomes of patients with perianal Paget's disease.

DESIGN: Institutional databases were queried for all of the cases of perianal Paget's disease at Memorial Sloan-Kettering Cancer Center between 1950 and 2011. Clinicopathologic factors were investigated for association with recurrence and survival.

SETTINGS: The study was conducted at a tertiary care center.

PATIENTS: Sixty-five patients with perianal Paget's disease were included in the study (35 women [54%]; median age at diagnosis, 66 years [range, 60-72 years]; and 41 with invasive disease/24 with noninvasive disease). A total of 56% with invasive disease were men.

MAIN OUTCOME MEASURES: Measures included median follow-up, disease status, local and distant recurrence, sites of recurrence, disease-specific survival, overall survival, and treatment modality.

RESULTS: A total of 95% with invasive disease and 87% with noninvasive disease were symptomatic at presentation. The most common symptoms were pruritus and perianal bleeding. The duration of symptoms was longer in patients with invasive (12.0 months; range, 4.0-18.0 months) versus noninvasive (3.5 months; range, 1.0-10.0 months) disease. Synchronous malignancies unrelated to the primary disease were noted in 5 patients with invasive disease and 3 with noninvasive disease. Noninvasive disease was treated with a wide local excision and invasive disease with a wide local excision (n = 32, 78%) or abdominoperineal resection (n = 9, 22%). Forty-one patients (27 invasive and 14 noninvasive) required multiple operations for tumor clearance. In those with invasive disease, the median time to recurrence was 5 years, and the median tumor-specific survival rate was 10 years.

LIMITATIONS: This was a retrospective study, limited by selection bias.

CONCLUSIONS: Perianal Paget's disease is associated with nonspecific symptoms, frequently delaying diagnosis. Wide local excision is the treatment of choice if negative margins can be obtained. Abdominoperineal resection should be considered for invasive disease. Local recurrence is common; follow-up includes periodic proctoscopy and digital examination. Invasive disease can metastasize to distant sites; follow-up should include the examination of inguinal lymph nodes and the imaging of liver and lungs.