Extramammary Paget Disease

Funaro, Deana; Krasny, Mark; Lam, Christina; Désy, Delphine; Sauthier, Philippe; Bouffard, Danielle

doi:10.1097/lgt.0b013e31825f4b4f

Cited by 32 publications

(9 citation statements)

References 56 publications

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“…It commonly affects areas rich in apocrine sweat glands, including the vulva, perineal area, perianal area, scrotal area, and penile skin (1,2). EMPD typically affects Caucasian females and Asian males older than 60 years (3)(4)(5)(6)(7). Most EMPD tumors are restricted to the epidermis as in situ lesions, and they are associated with good prognosis because of their slow-growing nature (1,8).…”

Section: Introductionmentioning

confidence: 99%

Mucosal Invasion, but Not Incomplete Excision, Has Negative Impact on Long-Term Survival in Patients With Extramammary Paget’s Disease

et al. 2021

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BackgroundExtramammary Paget’s disease (EMPD) sometimes spreads from the skin to mucosal areas, and curative surgical excision of these areas is challenging. The aim of this study is to analyze the impact of mucosal involvement and surgical treatment on the survival of patients with EMPD.MethodsWe conducted a retrospective review of 217 patients with EMPD. We also assessed the associations between tumor involvement in boundary areas (anal canal, external urethral meatus, vaginal introitus), prognostic factors, and survival in 198 patients treated with curative surgery.ResultsOf 217 patients, 75 (34.6%) had mucosal boundary area involvement. Lesions in these areas were associated with frequent lymphovascular invasion (p = 0.042), lymph node metastasis (p = 0.0002), incomplete excision (p < 0.0001), and locoregional recurrence (p < 0.0001). Boundary area involvement was an independent prognostic factor associated with disease-specific survival, per multivariate analysis (HR: 11.87, p = 0.027). Incomplete excision was not significantly correlated with disease-specific survival (HR: 1.05, p = 0.96).ConclusionBoundary area tumor involvement was a major risk factor for incomplete excision, local recurrence, and poor survival outcomes. However, incomplete removal of primary tumors was not significantly associated with poor prognosis. A less invasive surgical approach for preserving anogenital and urinary functions may be acceptable as the first-line treatment for resectable EMPD.

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Section: Introductionmentioning

confidence: 99%

Mucosal Invasion, but Not Incomplete Excision, Has Negative Impact on Long-Term Survival in Patients With Extramammary Paget’s Disease

et al. 2021

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“…Extramammary Paget's disease most frequently arises in the anogenital area in elderly people and less commonly in the axillary area [2][3][4]. This disease typically affects Caucasian woman and Asian men older than 60 years [3][4][5][6]. Most EMPD tumors remain restricted to the epidermis as in situ lesions, and they carry a good prognosis because of their slow-growing nature [1,7].…”

Section: Introductionmentioning

confidence: 99%

NECTIN4 Expression in Extramammary Paget’s Disease: Implication of a New Therapeutic Target

Murata

Ito

Tanaka

et al. 2020

IJMS

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Extramammary Paget’s disease (EMPD) is a rare skin cancer arising in the anogenital area. Most EMPD tumors remain dormant as in situ lesions, but the outcomes of patients with metastatic EMPD are poor because of the lack of effective systemic therapies. Nectin cell adhesion molecule 4 (NECTIN4) has attracted attention as a potential therapeutic target for some cancers. Urothelial cancer is one such cancer, and clinical trials of enfortumab vedotin, a drug-conjugated anti-NECTIN4 antibody, are ongoing. However, little is known regarding the role of NECTIN4 in EMPD. In this study, we conducted immunohistochemical analysis of NECTIN4 expression in 110 clinical EMPD samples and normal skin tissue. In normal skin, positive signals were observed in epidermal keratinocytes (particularly in the lower part of the epidermis), eccrine and apocrine sweat glands, inner and outer root sheaths, and matrix of the hair follicles. The most EMPD lesions exhibited strong NECTIN4 expression, and high NECTIN4 expression was significantly associated with increased tumor thickness, advanced TNM stage, and worse disease-specific survival. These results support the potential use of NECTIN4-targeted therapy for EMPD. Our report contributes to the better understanding of the pathobiology of NECTIN4 in the skin and the skin-related adverse effects of NECTIN4-targeted therapy.

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“…The prevalence of EMPD regarding gender differs between Western and Asian populations. In studies of Caucasian populations, EMPD has a female predominance, with male-to-female ratios ranging from 1:2 to 1:7 [1,[9][10][11][12]. In contrast, male predominance has been reported in Asian studies [3,13].…”

Section: Review 21 Epidemiologymentioning

confidence: 95%

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Ishizuki

Nakamura

2021

Current Oncology

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Extramammary Paget’s disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.

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Extramammary Paget Disease

Abstract: Extramammary Paget disease is more commonly found on the vulva of older women and frequently recurs. Recurrence was not associated to margin status, which would support a more conservative therapeutic approach.

Cited by 32 publications

References 56 publications

Mucosal Invasion, but Not Incomplete Excision, Has Negative Impact on Long-Term Survival in Patients With Extramammary Paget’s Disease

Mucosal Invasion, but Not Incomplete Excision, Has Negative Impact on Long-Term Survival in Patients With Extramammary Paget’s Disease

NECTIN4 Expression in Extramammary Paget’s Disease: Implication of a New Therapeutic Target

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

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