Extramammary Paget Disease Treatment & Management

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 20, 2011
 

Chemotherapy

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

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.[10]

Next

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.[11]

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.[12, 13] 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.

Previous
Next

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.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Neil Sandhu, MD  Resident Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Neil Sandhu, MD is a member of the following medical societies: American Dermatological Association, American Medical Association, American Medical Student Association/Foundation, American Roentgen Ray Society, American Society of Dermatology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

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. Nov 2 2009;[Medline].

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

  3. Bagby CM, MacLennan GT. Extramammary Paget's disease of the penis and scrotum. J Urol. Dec 2009;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. Feb 2005;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. Mar 2010;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. Jan 4 2010;[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. Sep 2009;16(5):81-3. [Medline].

  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. Oct 15 2009;[Medline].

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

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

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

  12. 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. Feb 15 1991;67(4):933-8. [Medline].

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

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.