Extramammary Paget's disease secondary prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Secondary prevention strategies following extramammary Paget’s disease include an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.

• As some patients develop recurrences more than 15 years after initial treatment, follow up needs to be long term. Long-term follow up is necessary to exclude both local recurrence and the development of associated internal malignancies.^[1][2]
• It is suggested that follow up for perianal extramammary Paget’s disease (EMPD) should involve an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.^[3]
• Because of multifocality of lesions, local recurrences are common even when the margins of resection are negative.
• Vulval EMPD may be similarly followed up with regular inspection of the vulva, the use of punch biopsies to exclude invasive disease in any recurrent lesion and regular pelvic ultrasound scans and hysteroscopy.^[4]
• Some physicians recommend lifelong follow-up after radical treatment of perianal disease, and the control examinations include a complete physical examination, proctosigmoidoscopy, double-contrast bowel enema, and biopsy of the perianal region once a year.^[5]
• Patients with EMPD require follow-up examination every three months after surgery to assess recurrence. This routine follow-up should continue for at least 24 months, after which follow-up examinations may be done annually.

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