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5/1/2010

Management of Paget Disease of the Vulva

Author: Philip J. DiSaia, MD

Editor: Daniel Martingano, MD

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Extramammary Paget Disease (EMPD) of the vulva is a rare intra-epithelial adenocarcinoma  representing 1-2% of all vulvar cancers. Overall, EMPD accounts for approximately 7% of all Paget disease, with approximately 65% of all EMPD involving the vulva. Vulvar EMPD is more prevalent among Caucasian, menopausal patients, higher body mass index patient, and in those who have used hormone replacement therapy. Although vulvar EMPD is typically diagnosed at a locally-advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. 

Diagnosis is often delayed since patients may present without symptoms or with minor pruritic symptoms attributed to otherwise benign or transient conditions by both patient and provider. Physical exam usually demonstrates either vulvar skin that is red in appearance with a violaceous hue resembling an erythematous lesion, or with a red epithelium that is partially covered with a thin white coating classically described as a “cupcake frosting” appearance.

The diagnosis is confirmed by punch biopsy with identification of vacuolated Paget cells. Yet, histologic appearance alone may misdiagnose EMPD with similar appearing intraepidermal carcinomas, necessitating confirmation by specific immunohistochemical stains (e.g. cytokeratin 7).

Standard treatment is wide local excision with subsequent vulvar/perineal reconstruction often requiring a multidisciplinary effort with both gynecologic oncology and plastic surgery divisions. Inguinal lymphadenectomy is not necessary unless the final pathology reports invasion. Complete vulvectomy with skin graft may be required for extensive or recurrent disease.

Recurrence is common despite negative surgical margins and can be present at distant sites. Multiple local excisional procedures are often required over a 10–20-year period resulting in the risk of subsequent vulvar disfigurement impacted quality of life.

Medical therapies include topical imiquimod, fluorouracil, and bleomycin. Imiquimod can be safely used for the treatment of VPD, including micro-invasive disease, with the largest study noting a 43% response rate. Obliterative treatments include carbon dioxide laser and photodynamic therapy. Skin complications including pain, desquamation and ulceration are common among all such treatments.

Although adenocarcinomas of the rectum, colon, bladder, breast and uterus associated with vulvar EMPD are rare, it is advisable to conduct a further evaluation of the breast, genitourinary tract, and gastrointestinal tract in women with biopsy confirmed Paget Disease of the vulva to exclude concurrent malignancy, especially if Paget lesions are found on the urethra and perianal areas.

Further Reading:

Borella F, Preti M, Vieira-Baptista P, Pérez-López FR, Bertero L, Gallio N, Micheletti L, Benedetto C. Vulvar Paget's disease: outcomes of 51 patients treated with imiquimod cream. Maturitas. 2022 Sep;163:23-27. doi: 10.1016/j.maturitas.2022.05.010. Epub 2022 May 27. PMID: 35661559.

Caruso G, Barcellini A, Mazzeo R, Gallo R, Vitale MG, Passarelli A, Mangili G, Pignata S, Palaia I. Vulvar Paget's Disease: A Systematic Review of the MITO Rare Cancer Group. Cancers (Basel). 2023 Mar 16;15(6):1803. doi: 10.3390/cancers15061803. PMID: 36980691; PMCID: PMC10046936.

Nasioudis D, Bhadra M, Ko EM. Extramammary Paget disease of the vulva: Management and prognosis. Gynecol Oncol. 2020 Apr;157(1):146-150. doi: 10.1016/j.ygyno.2019.11.009. Epub 2019 Nov 25. PMID: 31780234.

Caruso G, Barcellini A, Mazzeo R, Gallo R, Vitale MG, Passarelli A, Mangili G, Pignata S, Palaia I. Vulvar Paget's Disease: A Systematic Review of the MITO Rare Cancer Group. Cancers (Basel). 2023 Mar 16;15(6):1803. doi: 10.3390/cancers15061803. PMID: 36980691; PMCID: PMC10046936.

Initial Approval May 2010. Revised September 2015. Revised January 2018. Revised July 2019; Revised March 2021; Revised November 2022; Reaffirmed September 2024

 

 

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The Society for Academic Specialists in General Obstetrics and Gynecology, Inc. (“SASGOG”) 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. SASGOG 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. SASGOG does not guarantee, warrant, or endorse the products or services of any firm, organization, or person. Neither SASGOG nor its 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 2024 The Society for Academic Specialists in General Obstetrics and Gynecology, Inc. All rights reserved. No re-print, duplication or posting allowed without prior written consent.

 

 

 

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

The Society for Academic Specialists in General Obstetrics and Gynecology, Inc. (“SASGOG”) 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. SASGOG 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. SASGOG does not guarantee, warrant, or endorse the products or services of any firm, organization, or person. Neither SASGOG nor its 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 2022 The Society for Academic Specialists in General Obstetrics and Gynecology, Inc. All rights reserved. No re-print, duplication or posting allowed without prior written consent.

 

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