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.
Dr Daniel Perez and colleagues reviewed the diagnosis, management, and outcomes of patients with perianal Paget’s disease.
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.
The research team conducted the study at a tertiary care center.
The team included 65 patients with perianal Paget’s disease in the study.
|95% with invasive disease were symptomatic at presentation|
|Diseases of the Colon & Rectum|
A total of 56% with invasive disease were men.
The team's main outcomes included median follow-up, disease status, local and distant recurrence, sites of recurrence, disease-specific survival, overall survival, and treatment modality.
A total of 95% with invasive disease and 87% with noninvasive disease were symptomatic at presentation.
The team found that the most common symptoms were pruritus and perianal bleeding.
The duration of symptoms was longer in patients with invasive versus noninvasive 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 or abdominoperineal resection.
The team observed that 41 patients 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.
Dr Perez's team concludes, "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, and 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."