BACKGROUND: Extramammary Paget's disease is a rare cutaneous adenocarcinoma that occurs in an apocrine gland distribution mainly in the anogenital region. OBJECTIVE: To formulate treatment recommendations for this rare disease, we examined clinical and follow-up data of patients with it. METHODS: A retrospective review is given about the treatment and outcome for 95 patients at Mayo Clinic, Rochester, Minnesota, and Scottsdale, Arizona, between 1976 and 2001. The literature regarding diagnosis and treatment of this disease is also reviewed. RESULTS: Of the 95 patients, 86 had primary disease and 9 had recurrent disease. At mean follow-up (wide excision, 65 months; Mohs surgery, 24 months), disease had recurred in 18 of 83 (22%) who underwent standard wide excision, compared with recurrence in 1 of 12 (8%) who had the Mohs micrographic excision. CONCLUSION: Mohs micrographic surgery compares favorably with wide excision. Intraoperative immunostaining with cytokeratin 7 is helpful in delineating disease, as are preoperative scouting biopsies and photodynamic diagnosis.
BACKGROUND: Extramammary Paget's disease is a rare cutaneous adenocarcinoma that occurs in an apocrine gland distribution mainly in the anogenital region. OBJECTIVE: To formulate treatment recommendations for this rare disease, we examined clinical and follow-up data of patients with it. METHODS: A retrospective review is given about the treatment and outcome for 95 patients at Mayo Clinic, Rochester, Minnesota, and Scottsdale, Arizona, between 1976 and 2001. The literature regarding diagnosis and treatment of this disease is also reviewed. RESULTS: Of the 95 patients, 86 had primary disease and 9 had recurrent disease. At mean follow-up (wide excision, 65 months; Mohs surgery, 24 months), disease had recurred in 18 of 83 (22%) who underwent standard wide excision, compared with recurrence in 1 of 12 (8%) who had the Mohs micrographic excision. CONCLUSION: Mohs micrographic surgery compares favorably with wide excision. Intraoperative immunostaining with cytokeratin 7 is helpful in delineating disease, as are preoperative scouting biopsies and photodynamic diagnosis.