A Case of Chronic and Relapsing Paget Disease of the Vulva

Bouceiro-Mendes, Rita; Mendonça-Sanches, Maria; Soares‐de‐Almeida, Luís; Correia-Fonseca, Isabel

doi:10.1055/s-0039-1687861

Cited by 4 publications

(4 citation statements)

References 27 publications

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“…High recurrence rates are typical, and overtreatment should be avoided. 18 In our series melanoma accounted for only 0.9% (2/52) of malignant neoplasms which is inferior to the 3%-10% reported rates. 9,11 It is more common is postmenopausal women.…”

Section: Most Frequently Biopsied Malignant Vulvar Neoplasmscontrasting

confidence: 73%

Vulvar Diseases that Required a Biopsy: A Retrospective Study

Bouceiro-Mendes

Mendonça-Sanches

Soares‐de‐Almeida

et al. 2021

SPDV

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Introduction: The vulvar area may be affected by many noninfectious conditions with similar clinical appearance, requiring a cutaneous biopsy. Our goal was to characterize the noninfectious vulvar diseases that required a biopsy in a southwestern Europe Central Hospital during a 10-year period. Methods: A retrospective study of all the noninfectious vulvar diseases with histological confirmation diagnosed in our institution was conducted between January 1, 2008 and December 31, 2017. Results: The sample included a total of 323 biopsies from 317 patients, aged between 11 and 98 years (mean age of 54.2 years). A total of 36 vulvar diseases was identified. Neoplastic conditions were the most frequently found, particularly melanotic macules (22.3%). The most frequent malignant tumor was vulvar intraepithelial neoplasia (6.2%) and squamous cell carcinoma (5.6%). The most common dermatosis was lichen sclerosus (12.7%). Conclusion: Neoplasms were the most frequently diagnosed conditions affecting the vulvar area that required a biopsy. Ruling out malignancy was also the main reason to perform a biopsy. This study highlights the variety of noninfectious diseases that may affect the vulva and require a biopsy. Since vulvar diseases may be serious and carry high levels of patient distress a correct understanding of these conditions is crucial.

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Section: Most Frequently Biopsied Malignant Vulvar Neoplasmscontrasting

confidence: 73%

Vulvar Diseases that Required a Biopsy: A Retrospective Study

Bouceiro-Mendes

Mendonça-Sanches

Soares‐de‐Almeida

et al. 2021

SPDV

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“…Of the 34 articles included in this review, 10 were case reports [ 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ], 10 case series (4 of them reporting 2 cases, 2 with 3 cases, 1 with 4 cases, 1 with 6 cases, 1 with 10 cases, and the last one reporting 44 cases) [ 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ], 9 retrospective studies [ 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 ], 2 multicentric retrospective studies [ 40 , 41 ], 2 multicenter prospective studies [ 42 , 43 ] and 1 prospective study [ 44 ]. Women, 3244 in number, with primary or recurrent EMPD were included.…”

Section: Resultsmentioning

confidence: 99%

“…Regarding the type of treatment, 390 patients were treated with medical or other conservative treatments while 2802 patients were treated surgically; specifically, 182 patients analyzed were treated with topical 5% imiquimod cream [ 11 , 12 , 13 , 14 , 15 , 16 , 17 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 29 , 38 , 39 , 40 , 42 , 43 ], 72 with immunotherapy [ 36 ], 52 with radiotherapy [ 19 , 20 , 24 , 31 , 33 , 36 ], 32 with laser or other ablative methods [ 18 , 35 , 36 , 38 , 41 , 44 ], 23 patients with chemotherapy [ 14 , 36 ], 14 with topical 16% methyl aminolevulinate (MAL) + photodynamic therapy (PDT) [ 17 , 41 ], 10 with Fractional CO 2 Laser irradiation followed by photodynamic therapy [ 44 ], 3 with 5-fluorouracil [ 35 , 37 ] and 2 patients underwent CO 2 laser excision [ 34 ]. Among patients who underwent surgery, approximately 40% of them underwent local surgical excision [ 19 , 36 , 38 ], 31% simple partial/total vulvectomy [ 19 , 24 , 29 , 31 , 32 , 33 ,…”

Section: Resultsmentioning

confidence: 99%

Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light!

Corte

Cafasso

Conte

et al. 2023

JPM

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Background: Vulvar Paget’s disease (VPD) is defined as a neoplasm of epithelial origin, mostly in postmenopausal women. Due to the extreme rarity of VPD, limited data about recommended treatment options are available. Surgical excision has been the treatment of choice although in the recent decade medical treatments have been proposed. Methods: A systematic computerized search of the literature was performed in the main electronic databases (MEDLINE, EMBASE, Web of Science, PubMed, and Cochrane Library), from 2003 to September 2022, in order to analyze all medical and surgical strategies used for the treatment of VPD. Results: Thirty-four articles were included in this review with findings as follows: 390 patients were treated with medical or other conservative treatment while 2802 patients were treated surgically; 235/434 (54%) patients had a complete response, 67/434 (15%) a partial response, 10/434 (2.3%) a stable disease, 3/434 (0.7%) disease progress, 3/434 (0.7%) died of the disease, 55/434 (13%) died of other causes during follow up while 7/434 (1.6%) had to stop topical treatments with 5% imiquimod cream because of side effects; 239/434 patients (55%) had a recurrence and 11/434 (2.5%) were lost to follow-up. The length of follow-up was variable, according to the different studies analyzed. Conclusion: VPD is a chronic disease with a high recurrence rate and low mortality. There are no significant differences in recurrence rates in patients who undergo surgery and those who do not and the margin status at the time of primary surgery and recurrence. Several surgical and medical approaches providing both local control of the disease and minimal tissue damage have been developed. Clock mapping, a recent preoperative vulvo-vaginal workup tool, can predict the invasiveness and the extension of VPD. However, to date, due to the different treatment options available and in the absence of a global consensus, it is critical to tailor treatments to individual patient characteristics and biopsy histopathologic findings, to ensure the best type of therapy.

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“…This makes margin control hard to achieve. The preferred surgical method uses Mohs micrographic surgery to avoid falsenegative free margins in the results of the frozen section, as in our case (15). Surgery varies from local excision to radical vulvectomy, with a high risk of recurrence regardless of the margin status which then leads to increasingly mutilating surgery, and thus plastic reconstructive surgery is often needed to cover the tissue loss, as in our case.…”

Section: Discussionmentioning

confidence: 96%

Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature

Bouhani¹,

Zemni²,

Fertani³

et al. 2019

JMMS

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Vulvar Paget's disease is an uncommon adenocarcinoma of the female genital tract, occuring more often in postmenopausal Caucasian women and accounting for 1-2% of all vulvar malignancies. A 39-year-old female patient was examined for the erythematous and pruritic vulvar lesion, with biopsy and histological examination revealing vulvar Paget's disease. We did not diagnose any associated neoplasm. The patient underwent four surgical resections for relapsed disease, varying from local excision to extended excision, including radical vulvectomy, with esthetic coverage. The margins were involved. Due to the extended target volumes, radiotherapy was rejected and the patient underwent a local topical therapy with Imiquimod 5%. The patient was free of disease, with three years of regular follow-ups. This case was challenging due to the long recurrence of the disease for approximately 26 years and due to the therapeutics issues that occurred.

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A Case of Chronic and Relapsing Paget Disease of the Vulva

Cited by 4 publications

References 27 publications

Vulvar Diseases that Required a Biopsy: A Retrospective Study

Vulvar Diseases that Required a Biopsy: A Retrospective Study

Medical and Surgical Strategies in Vulvar Paget Disease: Let’s Throw Some Light!

Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature

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