Perianal Extramammary Paget’s Disease: Diagnosis, Treatment and Prognosis

Background: To make an update on the state of knowledge on perianal extramammary Paget’s disease. Case presentation: We report one case as an illustration of this rare, slow-growing and occasionally metastasizing entity. The standard treatment is a local surgical excision. Radiation therapy is recommended as primary treatment in inoperable patients, and as alternative to surgery for recurrence disease previously excised or in case of mutilating surgery. Conclusions: Definitive conclusion cannot be drawn. Clinical trials are required to test whether radiation therapy is advantageous as primary treatment approach, compared to surgery.