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Extramammary Paget Disease Treatment & Management

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 03, 2014
 

Chemotherapy

Three separate reports describe successful treatment of extramammary Paget disease (EMPD) with 5-fluorouracil,[7] imiquimod,[8, 9] and a combination of paclitaxel and trastuzumab.[10]

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.[11]

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Surgical Excision

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.[12]

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.

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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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Contributor Information and Disclosures
Author

Neil Sandhu, MD Dermatologist (Medical/Cosmetics) and Mohs Surgeon, Gulf Coast Dermatology

Neil Sandhu, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Marjan Garmyn, MD, PhD Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Joseph L Wilde, MD Chief, Department of Dermatology, Vicenza Army Health Center, Italy

Joseph L Wilde, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

References
  1. Roy J, Mirnezami A, Gatt M, Sasapu K, Scott N, Sagar P. A rare case of Paget's disease in a retrorectal dermoid cyst. Colorectal Dis. 2009 Nov 2. [Medline].

  2. Chanda JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985 Dec. 13(6):1009-14. [Medline].

  3. Bagby CM, MacLennan GT. Extramammary Paget's disease of the penis and scrotum. J Urol. 2009 Dec. 182(6):2908-9. [Medline].

  4. Cho SB, Yun M, Lee MG, Chung KY. Variable patterns of positron emission tomography in the assessment of patients with extramammary Paget's disease. J Am Acad Dermatol. 2005 Feb. 52(2):353-5. [Medline].

  5. D'Agostino M, Cinelli C, Willard R, Hofmann J, Jellinek N, Robinson-Bostom L. Epidermotropic Merkel cell carcinoma: a case series with histopathologic examination. J Am Acad Dermatol. 2010 Mar. 62(3):463-8. [Medline].

  6. Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The Role of Immunohistochemistry in Discriminating Primary From Secondary Extramammary Paget Disease. Am J Dermatopathol. 2010 Jan 4. [Medline].

  7. Beleznay KM, Levesque MA, Gill S. Response to 5-fluorouracil in metastatic extramammary Paget disease of the scrotum presenting as pancytopenia and back pain. Curr Oncol. 2009 Sep. 16(5):81-3. [Medline]. [Full Text].

  8. Sendagorta E, Herranz P, Feito M, et al. Successful treatment of three cases of primary extramammary Paget's disease of the vulva with Imiquimod - proposal of a therapeutic schedule. J Eur Acad Dermatol Venereol. 2009 Oct 15. [Medline].

  9. Marchitelli C, Peremateu MS, Sluga MC, et al. Treatment of primary vulvar paget disease with 5% imiquimod cream. J Low Genit Tract Dis. 2014 Oct. 18(4):347-50. [Medline].

  10. Takahagi S, Noda H, Kamegashira A, et al. Metastatic extramammary Paget's disease treated with paclitaxel and trastuzumab combination chemotherapy. J Dermatol. 2009 Aug. 36(8):457-61. [Medline].

  11. Cecchi R, Pavesi M, Bartoli L, Brunetti L, Rapicano V. Perineal extramammary Paget disease responsive to topical imiquimod. J Dtsch Dermatol Ges. 2010 Jan. 8(1):38-40. [Medline].

  12. Appert DL, Otley CC, Phillips PK, Roenigk RK. Role of multiple scouting biopsies before Mohs micrographic surgery for extramammary Paget's disease. Dermatol Surg. 2005 Nov. 31(11 Pt 1):1417-22. [Medline].

  13. Coldiron BM, Goldsmith BA, Robinson JK. Surgical treatment of extramammary Paget's disease. A report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision. Cancer. 1991 Feb 15. 67(4):933-8. [Medline].

  14. Hendi A, Brodland DG, Zitelli JA. Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol. 2004 Nov. 51(5):767-73. [Medline].

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Photomicrograph of malignant melanoma in situ of skin displays prominent intraepidermal pagetoid spread. Note that melanoma cells are present in all layers of epidermis, mostly in single units. Cytoplasm of melanoma cells is vacuolated. Moderate upper dermal chronic inflammatory infiltrate is present (hematoxylin-eosin, original magnification ×250). S-100 protein and homatropine methylbromide immunostains are positive in melanoma cells, whereas carcinoembryonic antigen is negative. No epithelial mucin is seen in these tumor cells.
 
 
 
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