Purpose: Perianal Paget's disease is a rare intraepithelial adenocarcinoma of the perianal skin and the second most common localization of extramammary Paget's disease. This study was designed to evaluate long-term outcomes in patients with perianal Paget's disease.
Methods: We identified patients who were treated for perianal Paget's disease between 1981 and 2013. Patient demographics, family history, associated malignancies, treatments, histopathological features, need for re-operations, and long-term outcomes were documented.
Results: Our study cohort consisted of 15 male and 10 female patients with a median age of 67 (40-83) years. Four patients had concurrent anorectal adenocarcinoma (two anal canal, two rectal) when perianal Paget's disease was diagnosed. Index operations performed were wide local excision (14 patients), local excision (five patients), abdominoperineal resection (four patients), and radiotherapy (two patients). Five patients developed invasive carcinoma (three anal canal, one vulvar, one perianal squamous cell carcinoma) during a median follow-up time of 60 (3-299) months. Thirteen patients were re-operated. Fifteen patients had a reconstructive procedure following excision. Overall survival was similar between the patients who were treated with wide local excision and local excision regardless of surgical margin status at index excision (P = 0.75).
Conclusions: Since there is a risk for developing invasive carcinoma in the anal canal and perineum in patients with perianal Paget's disease, close follow-up is needed after R0 or R1 excision. Physicians should be aware of the risk of cancers associated with perianal Paget's disease and should rule out them during management.
Keywords: Extramammary Paget’s disease; Intraepithelial adenocarcinoma; Long-term outcomes; Perianal Paget’s disease.