Vulvar squamous cell carcinoma and lichen sclerosus

Abstract

There are two clinicopathological types of vulvar squamous cell carcinoma, human papillomavirus (HPV)-positive and HPV-negative, which can be distinguished to some degree on routine histology. Human papillomavirus-positive carcinomas account for one-quarter to one-third of cases, occur in women on average 20 years younger than in HPV-negative, and are associated with multiple lower genital tract neoplasia. Human papillomavirus negative carcinoma is linked to lichen sclerosus. Of all carcinomas, 7-96% show lichen sclerosus in skin adjacent to the carcinoma, the majority being the first presentation of lichen sclerosus, and up to 5% of patients with lichen sclerosus develop carcinoma after long-term follow up. Where lichen sclerosus is associated with malignancy, it is often hyperplastic, may show a subtle form of intraepithelial neoplasia termed 'differentiated vulvar intraepithelial neoplasia', and may lose its pathognomonic oedematous-hyaline layer. The local additional factors causing lichen sclerosus to develop malignancy on the vulva are not known.

Long‐term management of vulval lichen sclerosus in adult women

“…3,10,11 The few studies of long-term management regimes could not answer the question whether treatment reduces the risk of SCC or progressive scarring. 10,12,13 Some authors have argued that long-term follow-up is unnecessary and represents an unreasonable burden on the health care system.…”

A cluster of vulvar cancer and vulvar intraepithelial neoplasia in young Australian Indigenous women

“…VIN and vulvar cancer occur in two distinctive clinicopathological groups [6,7]. In younger women (35-55 years of age), VIN and vulvar cancer are associated with persistent infection with oncogenic genotypes of HPV [8].…”

Paediatric vulval lichen sclerosus

“…Where LS is associated with malignancy, it is often hyperplastic, may show a subtle form of intraepithelial neoplasia termed 'differentiated vulval intraepithelial neoplasia', and may lose its pathognomonic oedematous-hyaline layer. 27 However, extragenital LS is not associated with malignancy. 28 The long-term risk of vulval malignancy for patients with LS in childhood and adolescence is unknown, but in adult women, studies conducted prior to the establishment of effective therapy have shown a 4-6% risk of developing a vulval SCC.…”