The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD) and the European Federation for Colposcopy (EFC) consensus statements on pre-invasive vulvar lesions

Abstract

The European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD), and the European Federation for Colposcopy (EFC) developed consensus statements on pre-invasive vulvar lesions in order to improve the quality of care for patients with vulvar squamous intraepithelial neoplasia, vulvar Paget disease in situ, and melanoma in situ. For differentiated vulvar intraepithelial neoplasia (dVIN), an excisional procedure must always be adopted. For vulvar high-grade squamous intraepithelial lesion (VHSIL), both excisional procedures and ablative ones can be used. The latter can be considered for anatomy and function preservation and must be preceded by several representative biopsies to exclude malignancy. Medical treatment (imiquimod or cidofovir) can be considered for VHSIL. Recent studies favor an approach of using imiquimod in vulvar Paget’s disease. Surgery must take into consideration that the extension of the disease is usually wider than what is evident in the skin. A 2 cm margin is usually considered necessary. A wide local excision with 1 cm free surgical margins is recommended for melanoma in situ. Following treatment of pre-invasive vulvar lesions, women should be seen on a regular basis for careful clinical assessment, including biopsy of any suspicious area. Follow-up should be modulated according to the risk of recurrence (type of lesion, patient age and immunological conditions, other associated lower genital tract lesions).

Identification of Risk Factors of Recurrence Among Patients With Vulvar Paget Disease Treated With Conservative Surgery

“…We conducted a retrospective study to identify patients who were diagnosed in our institution with noninvasive type Ia VPD 1 of cutaneous origin based on the Brown and Wilkinson classification 11 between 2008 and 2019. The study was approved by the institutional review board of our hospital before its onset (IRB approval number 182/2021).…”

“…Extramammary Paget disease is a rare form of superficial skin cancer. It is encountered in skin areas with abundant apocrine sweat glands and is characterized by the presence of cells with large pleomorphic nuclei that initially form in the lower layers of the epidermis, without invading the dermis, but as the disease progresses, they tend to involve the entire thickness of the epidermal layer 1 . Vulvar Paget disease (VPD) represents most extramammary lesions accounting for approximately 1% to 2% of all tumors of the female genital tract 2–4 .…”

“…Currently, VPD is treated with surgery that consists mainly of local excision of the lesion aiming to obtain at least macroscopically negative margins. Radical surgery should not be used in the absence of invasive features as the disease tends to recur in more than one third of cases 1,7,8 . A recent randomized trial suggested that topical application of imiquimod may be an equally effective and safe alternative to surgical excision 9 …”

“…It is encountered in skin areas with abundant apocrine sweat glands and is characterized by the presence of cells with large pleomorphic nuclei that initially form in the lower layers of the epidermis, without invading the dermis, but as the disease progresses, they tend to involve the entire thickness of the epidermal layer. 1 Vulvar Paget disease (VPD) represents most extramammary lesions accounting for approximately 1% to 2% of all tumors of the female genital tract. [2][3][4] The estimated incidence in Europe is approximately 0.7 cases per 100,000 women, 4,5 Its diagnosis may be difficult and the most prominent symptoms include pruritus and local pain, while macroscopically the lesion is usually an asymmetrical, red, and white eczematoid pruritic lesions or as an erythematous plaque with typical white scaling on the vulva, which progressively expands with a slow rate.…”

“…Radical surgery should not be used in the absence of invasive features as the disease tends to recur in more than one third of cases. 1,7,8 A recent randomized trial suggested that topical application of imiquimod may be an equally effective and safe alternative to surgical excision. 9 Given that multiple recurrences are rather frequent, regardless of the treatment modality that is used, 10 factors that contribute to their development should be thoroughly evaluated.…”