Paget disease of the vulva

Abstract

In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin disease, most commonly found in postmenopausal Caucasian women. The underlying cause of VPD remains not well understood. VPD is rarely associated with an underlying urogenital, gastrointestinal or vulvar carcinoma. In approximately 25% of the cases, VPD is invasive; in these cases, the prognosis is worse than in non-invasive cases. Recurrence rates in invasive VPD are high: 33% in cases with clear margins, and even higher when surgical margins are not clear, regardless of invasion. Historically, surgical excision has been the treatment of choice. Recent studies show that imiquimod cream may be an effective and safe alternative.

Extramammary Paget Disease of the Vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation

“…It is characterized by the presence of discohesive so-called Paget cells that tend to cluster in epithelial nests, but may also be interspersed throughout the epithelium. 1 The reported incidence of EMPD is 0.7 cases per 100,000 women in Europe 1 and there is no consensus on the incidence in the US population. Extramammary Paget disease may invade the dermis, though this occurrence is rare with invasive EMPD representing less than 2% of all vulvar malignancies.…”

“…Extramammary Paget disease may invade the dermis, though this occurrence is rare with invasive EMPD representing less than 2% of all vulvar malignancies. 1 Extramammary Paget disease is also associated with vulvar adenocarcinoma, as well as malignancies outside the vulva in 11% to 54% of cases. 1Y3 These may include breast, intestinal, and urologic malignancies.…”

“…1Y3 These may include breast, intestinal, and urologic malignancies. 1,4 The American Congress of Obstetricians and Gynecologists recommends that in patients with biopsyconfirmed Paget disease, there should be further evaluation of the breast, genitourinary tract, and gastrointestinal tract. 4 Extramammary Paget disease is challenging for patients and practitioners given the limited available data to guide treatment and high recurrence rates.…”

The Role of Radiotherapy in Extramammary Paget Disease: A Systematic Review

“…Surgery is the recommended therapy for resectable and localized disease, being associated to a low rate of local recurrences. 5 However, several other local treatments such as radiotherapy (RT), topical imiquimod cream, photodynamic or laser therapy, and chemotherapy 6,7 have been used, but available evidences have several limitations. Most articles report small retrospective studies, referring to patients treated decades ago with large heterogeneity in terms of RT dose and technique.…”

Reviewing vulvar Paget’s disease molecular bases. Looking forward to personalized target therapies: a matter of CHANGE

“…Conventional management primarily provides a surgical approach, which is burdened by high rates of complications and local recurrences, varying from 34% to 56% 3. Non-surgical alternatives (radiotherapy, photodynamic therapy, CO2 laser ablation, imiquimod 5% cream) show relatively unsuccessful outcomes.…”