Back to Search Results

Management of Paget Disease of the Vulva

5/1/2010 - Philip J. DiSaia, MD

Editor: Kenneth L. Noller, MD

 

Extramammary Paget Disease (EMPD) of the vulva is a rare intra-epithelial adenocarcinoma of the vulvar skin presenting mostly commonly with pruritus. Unfortunately, diagnosis is often delayed as both patients and physicians dismiss the itching as local irritation from garments or other irritants. There are two typical presentations.  The vulvar skin can be red, occasionally with violaceous hue (erythematous lesion). The red skin can be partially covered with a thin white coating with a “cupcake frosting” appearance (eczematoid lesion).

The diagnosis is made by biopsy with identification of vacuolated Paget cells. Immunohistochemistry is necessary to exclude vulvar intraepithelial neoplasia or melanoma.

Standard treatment is wide local excision. Since invasion to structures below the dermis is rare, inguinal lymphadenectomy is not necessary unless the final pathology reports invasion. Very rarely, complete vulvectomy with skin graft may be required for extensive disease.

Many clinicians perform frozen section margin analysis at the time of surgery and extend the resection until all margins are negative. Because of its multifocal and multicentric nature, recurrence is common even when the surgical margins are clear. The disease process appears to have the ability to skip over apparent normal skin and can recur at sites distant to the surgical site. For this reason, patients must be monitored closely and suspicious areas biopsied. In many patients, multiple local excisions are performed over a period of 10-20 years with the risk of vulvar disfigurement, impacting quality of life.

Although there are multiple case reports of alternative therapies for EMPD, none have been validated through randomized clinical trials. Carbon dioxide laser therapy has been used. Gauging the depth of treatment can be challenging and may result in repeat interventions. Photodynamic therapy uses a tumor sensitizing agent in combination with appropriate light wavelengths to destroy lesions, but can be problematic. More recently, Imiquimod, an immune modulator, has been successfully employed, typically used as a 5% cream 3-4 times weekly for 6 months. Patients should be monitored for adverse events, including skin irritation, erosion, pain or ulceration, with dosing adjusted accordingly.

Other therapies include radiotherapy and topical chemotherapeutic agents such as fluorouracil and bleomycin. Skin complications including pain, desquamation and ulceration are quite common. Regardless of treatment modality, reported treatment response ranges from 14%-100% with 0-67% recurrences.

Older literature reports adenocarcinomas of the rectum, colon, bladder, breast and uterus associated with Paget Disease of the vulva. However, more recent studies have found a very low frequency of simultaneous lesions. ACOG recommends further evaluation of the breast, genitourinary tract, and gastrointestinal tract in women with biopsy confirmed Paget Disease of the vulva.  Paget lesions around the urethra and perianal areas require careful evaluation for bladder or rectal tumors.

 

Further Reading:

Eddey KA, Allan E, Murdoch JB, et al. Interventions for the treatment of Paget’s disease of the vulva. Cochrane Database Syst Rev. 2013 Oct 26;(10):CD009245. doi: 10.1002/14651858.CD009245.pub2.

Machida H, Moeini A, Roman LD, Et al. Effects of Imiquimod on vulvar Paget’s disease: A systematic review of literature. Gynecol Oncol 2015;139:165-71.

ACOG Practice Bulletin No. 93, Diagnosis and management of vulvar skin disorders. American College of Obstetricians and Gynecologists. PMID: 18448767 DOI: 10.1097/AOG.0b013e31817578ba

 

Initial Approval May 2010. Revised , September 2015, January 2018.

 

********** Notice Regarding Use ************

The Foundation for Exxcellence in Women’s Health, Inc (“Foundation”) is committed to accuracy and will review and validate all Pearls on an ongoing basis to reflect current practice.

This document is designed to aid practitioners in providing appropriate obstetric and gynecologic care. Recommendations are derived from major society guidelines and high quality evidence when available, supplemented by the opinion of the author and editorial board when necessary. It should not be construed as dictating an exclusive course of treatment or procedure to be followed.

Variations in practice may be warranted when, in the reasonable judgment of the treating clinician, such course of action is indicated by the condition of the patient, limitations of available resources, or advances in knowledge or technology. The Foundation reviews the articles regularly; however, its publications may not reflect the most recent evidence. While we make every effort to present accurate and reliable information, this publication is provided “as is” without any warranty of accuracy, reliability, or otherwise, either express or implied. The Foundation does not guarantee, warrant, or endorse the products or services of any firm, organization, or person. Neither the Foundation, the ABOG, SASGOG nor their respective officers, directors, members, employees, or agents will be liable for any loss, damage, or claim with respect to any liabilities, including direct, special, indirect, or consequential damages, incurred in connection with this publication or reliance on the information presented.

 

Copyright 2018 - The Foundation for Exxcellence in Women's Health, Inc. All rights reserved. No publication, reuse or dissemination allowed without written permission.

Visit us at: www.exxcellence.org

 

 

 

Back to Search Results