BACKGROUND: Perianal Paget's disease was first described in 1893. Since then, fewer than 300 cases have been reported in the literature. It might be associated with an underlying malignancy. Most of the patients are treated with surgical excision, which is often mutilating and of variable efficacy. In the present report, we describe the case of an 80-year-old woman with perianal Paget's disease. She was treated at our institution with radiotherapy with curative intent. The pertinent literature is reviewed, and the controversies in diagnosis and management are discussed. CONCLUSIONS: Our single-case experience supports the opinion that radiotherapy is a viable treatment option with curative potential for patients with perianal Paget's disease. For the treatment of in situ disease, hypofractionated regimens to a total dose of 40-44 Gy and orthovoltage X-ray beams are recommended.
BACKGROUND: Perianal Paget's disease was first described in 1893. Since then, fewer than 300 cases have been reported in the literature. It might be associated with an underlying malignancy. Most of the patients are treated with surgical excision, which is often mutilating and of variable efficacy. In the present report, we describe the case of an 80-year-old woman with perianal Paget's disease. She was treated at our institution with radiotherapy with curative intent. The pertinent literature is reviewed, and the controversies in diagnosis and management are discussed. CONCLUSIONS: Our single-case experience supports the opinion that radiotherapy is a viable treatment option with curative potential for patients with perianal Paget's disease. For the treatment of in situ disease, hypofractionated regimens to a total dose of 40-44 Gy and orthovoltage X-ray beams are recommended.