Perianal Paget's Disease: Presentation of Twelve Cases and a Literature Review

Purpose. Perianal Paget's disease (PPD) is a rare intraepithelial adenocarcinoma. To date, ~200 cases have been reported, representing ~20% of all extramammary Paget's disease cases. The standard treatment is wide local excision, although PPD has a high recurrence rate. Here, we review our management for PPD and review the currently recommended treatment approaches. Methods. In this study, we reviewed 12 patients diagnosed with PPD between 1995 and 2014. Clinical data including age, sex, symptoms, symptom duration, histopathology, treatment modality, recurrence, follow-up duration, and survival outcome were evaluated. We also reviewed the published literature from 1990 to 2014 to identify the treatment strategies used in 213 cases of PPD. Results. Twelve patients with in situ disease with a median age of 70.8 (65-76) years, including 3 female patients (25%), were diagnosed with PPD. Eleven patients underwent surgery. The mean follow-up time was 92.1 (36-200) months. One patient died from liver cancer and bone metastasis, and the remaining patients are alive; 10 are disease-free and 1 experienced postsurgical local recurrence. This patient refused extensive radical excision, so radiotherapy (32 Gy in 16 fractions) was administered and the patient remains disease-free after 4 years. Conclusion. Surgery is the mainstay treatment for PPD. Nonsurgical modalities could be considered an alternative to surgery for those with noninvasive PPD who refuse radical surgery or who are medically unfit for surgery. Further research and follow-up are needed to compare the effectiveness of surgical and nonsurgical therapeutic modalities for PPD.