Extramammary Paget Disease Treatment & Management
- Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD more...
Specifically, imiquimod 5% cream applied 3 times weekly for 16 weeks induced complete resolution in a patient with perineal EMPD. Topical imiquimod is considered a possible treatment option, especially when surgery is a challenge or contraindicated. However, more studies are needed to confirm the use of topical therapies for patients with EMPD.
Margin-controlled surgical excision of all the involved epidermis is the most effective treatment. EMPD extends beyond the visibly involved margins. Obviously involved skin should be examined by using transverse frozen sections or serial vertical sections (see Workup). Multiple scouting biopsies performed before surgery may aid in planning a more precise initial excision.
Multifocal disease is a challenge for any surgical method that relies on contiguous tumor spread for effective margin control—even micrographic surgery. However, Mohs micrographic surgery offers lower recurrence rates after excision of primary tumors with a smaller margin of normal skin removed.[13, 14] The recurrence rate of primary tumors after standard surgical excision is 30-60%. The rate after excision with Mohs micrographic surgery is 8-26%. The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up.
Consultations and Long-Term Monitoring
Depending on the anatomic location of EMPD, treatment should be coordinated with an appropriate surgical subspecialist (eg, a urologist, a colorectal surgeon, or a gynecologist). Optimally, the consultant would have some experience treating this specific condition. Further consultation with a radiologist and a gastroenterologist may also be required to order appropriate screening examinations for internal malignancy.
Patients with EMPD require follow-up examination every 3 months after surgery to assess possible recurrence. This routine should continue for at least 24 months, after which time examinations may be done annually. Consider repeating other endoscopic or imaging studies on a regular basis according to the specific recommendations of the consultants.
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