Pre-malignant vulval disorders (original) (raw)
Related papers
Current Oncology
The aim of the present study was to evaluate the incidence of concomitant vulvar cancers or premalignant lesions in women surgically treated for extramammary Paget’s disease of the vulva (EMPDV) through a multicenter case series. The medical records of all women diagnosed with and treated for EMPDV from January 2010 to December 2020 were retrospectively analyzed. Women with EMPDV and synchronous vulvar cancer, vulvar intraepithelial neoplasia (VIN) and/or lichen sclerosus (LS) at the histology report were included in the study. A total of 69 women eligible for the present study were considered. Concomitant vulvar lesions occurred in 22 cases (31.9%). A total of 11 cases of synchronous VIN (50%) and 14 cases (63.6%) of concomitant LS were observed. One patient (4.5%) had synchronous vulvar SCC (FIGO stage 1B). Women with EMPDV and concomitant premalignant/malignant vulvar lesions had a significantly higher rate of invasive EMPDV and wider lesions with an extravulvar involvement. The ...
Management of lichen sclerosus and intraepithelial neoplasia of the vulva in the UK
Journal of the Royal Society of Medicine, 1996
Women with vulval intraepithelial neoplasia (VIN), lichen sclerosus (LS) and Paget's disease are referred either to gynaecologists or to dermatologists. We have ascertained the caseloads, referral patterns and treatment modalities used in the two specialties. A postal questionnaire was sent to 540 consultant gynaecologists and 225 consultant and senior registrar members of the British Association of Dermatologists. 350 gynaecologists and 161 dermatologists returned completed questionnaires. The workload of LS and Paget's disease was evenly distributed, with 54% of dermatologists and 58% of gynaecologists seeing more than six cases of LS per annum and less than 1% seeing more than five cases of Paget's disease. 92% of responding gynaecologists saw at least one case of VIN per year whereas 43% of dermatologists saw no cases. Patients with VIN and Paget's were referred to gynaecologists for treatment by 66% of dermatologists. Both groups are equally prepared to treat LS...
Vulvar lichen sclerosus, premalign inflammatory dermatosis
Gineco.eu, 2015
Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition classified as precancerous dermatosis of the genital mucosa, associated with a risk of progression to in situ, then invasive vulvar squamous cell carcinomas. Although the etiology of this disease has not been determined, the factors involved in its pathogenesis are genetic, autoimmune, hormonal, infectious as well as environmentally-mediated. Given the risk of malign progression, these type of skin lesions have to be carefully watched by regular clinical exams, and a biopsy of the affected genital mucosa should be carried out whenever dysplasia or malign transformation is suspected. Vulvar carcinogenesis is a multistage process, regardless of the type (i.e. differentiated or not) of intraepithelial vulval neoplasia (IVN), which includes human papilloma viruses (HPV)-negative immune-mediated inflammatory disease, mainly the VLS. The involvement of HPV as main trigger in IVN has been clearly confirmed. The present paper comprises a synthetic review of the available literature on this borderline type of disease referred to as VLS, and looks at the mainly clinical and diagnostic aspects that need to be observed by the clinicians. Considering the potential for progression of the disease, it is of foremost importance to diagnose VLS in an early stage, to closely follow up with the gynecologist and dermatologist, while carefully and steadily monitoring these lesions and to initiate the appropriate disease-specific treatment.
Non-neoplastic epithelial disorders of the vulva
American family physician, 2008
Lichen sclerosus, lichen planus, and lichen simplex chronicus are three of the most common non-neoplastic epithelial disorders of the vulva. Lichen sclerosus is characterized by intense vulvar itching and can affect men and women of all ages, but it manifests most commonly in postmenopausal women. Patients with lichen sclerosus have an increased risk of developing squamous cell carcinoma, and they should be monitored for malignancy. Lichen planus is an inflammatory autoimmune disorder that can affect the vulva and the vagina; it peaks in incidence between ages 30 and 60. There are three clinical variants of lichen planus affecting the vulva: erosive, papulosquamous, and hypertrophic. Lichen simplex chronicus is caused by persistent itching and scratching of the vulvar skin, which results in a thickened, leathery appearance. It is thought to be an atopic disorder in many cases and may arise in normal skin as a result of psychological stress or environmental factors. Definitive diagno...
Expert Review of Obstetrics & Gynecology, 2013
Historical notes Lichen sclerosus (LS) was first described at the end of the 19th century; it is usually attributed to Hallopeau, a French author who, in 1887, referred to it as 'lichen plan scléreux', considering it an atrophic variant of lichen planus [1]. However, this may not have been the first description of LS; a case of vulvar and oral 'ichthyosis' was in fact described in 1875 by Weir [2]. In 1892, Darier reported its histologic features, again using the French nomenclature of 'lichen plan scléreux' to define the disease [3]. Since then, a heterogeneous variety of different definitions have been used to describe this condition, such as kraurosis vulvae, leukoplakia, weissflecken dermatose, hypoplastic dystrophy, LS and atrophicus. In 1976, Friedrich argued that LS was more ' dystrophic' than 'atrophic' and proposed the removal of 'et atrophicus' from the nomenclature [4]. In the same year, the term LS was formally adopted by the newly formed International Society for the Study of Vulvar Diseases. Since then, a significant and very useful amount of work has been carried out by the International Society for the Study of Vulvar Diseases-renamed the International Society for the Study of Vulvovaginal Diseases-to clarify the pathogenesis, epidemiology, clinical patterns and therapy of this condition. Epidemiology The exact prevalence and incidence of LS still remain unknown, with very few studies existing. This is probably due to a fragmented approach in diagnosing and treating patients, since its management is frequently shared among multiple specialties, including dermatology, gynecology, urology, family medicine and pediatrics, and is very often without a multidisciplinary program. A study has reported that one out of 30 menopausal women suffered from LS [5], while another reports that the disease affects 1.7% of patients attending a general gynecology practice [6]. In 1971, Wallace observed that the incidence of vulvar LS varied between one in 300 and one in 1000 of all new patients referred to dermatology or gynecology out patient clinics [7]. In 1996, Thomas et al. performed a study on 355 women and found that 9% of cases occurred in prepubertal children, 42% during the reproductive years and 49% in women over 50 years of age [8]. Traditionally, this disorder is considered typical of the elderly population, yet it may affect women of all age groups, as well as children [9]. The incidence is lower in men than in women, although the same bimodal onset in the male population has been reported [10].
Vulvar Paget Disease: One Century After First Reported
Journal of Lower Genital Tract Disease, 2003
Objectives. To provide a critical assessment of the published literature on vulvar Paget disease and to allow individualized approaches to affected patients. Materials and Methods. A computerized search for studies published in the literature up to June 2002 was carried out using Ovidᮊ and Medline databases. We excluded single case reports, letters to editors, and abstracts. Results. Historical and epidemiological aspects of vulvar Paget disease are summarized. Clinical and histopathological data support a recent proposal to classify vulvar Paget disease into two categories, primary and secondary, with significant clinical and prognostic implications. The treatment for primary vulvar Paget disease is wide and deep surgical excision. Inguinofemoral lymphadenectomy is added in the management of invasive neoplasms. In the presence of secondary Paget disease, therapy must be directed toward treatment of associated carcinoma. Conclusions. The subclassification of vulvar Paget disease is essential for correct clinical management and treatment. Immunohistochemistry may assist in this important distinction. I
Extramammary Paget’s Disease Versus Lichen Sclerosus
Acta Medica Martiniana, 2016
Burning, itching and dyspareunia are typical symptoms of many genital diseases. These subjective complaints can be misdiagnosed because of different clinical presentations. We present a case report of a postmenopausal woman treated for genital warts over a period of three years followed by the development of leukoplastic lesions in the whitish area clinical classified as Lichen sclerosus (LS). Histology of this lesion revealed carcinoma in situ. After radical surgical removal, vulvar Paget's disease was histologically verified. LS and extramammary Paget´s disease (EMPD) belong to a group of uncommon dermatoses which mainly affect the skin of the genitals in postmenopausal women. Ulceration, erosions and leukoplastic lesions can signalize the development of squamous cell carcinoma in association of lichen sclerosus, on the other hand, they can be the sign of EMPD after a long period of time using different topical agents. The importance of reaching the correct diagnosis is essential and can influence current patient investigations and invasive or non-invasive treatment.
Vulvar Lichen Sclerosus and Neoplastic Transformation
Journal of Lower Genital Tract Disease, 2016
The aim of the study was to estimate the neoplastic potential of vulvar lichen sclerosus (VLS). This was a retrospective study of 976 women with VLS. We recorded age at diagnosis of VLS, length of follow-up, and type of neoplasia, categorized as the following: (1) vulvar intraepithelial neoplasia (VIN), further subdivided in differentiated VIN (dVIN) and high-grade squamous intraepithelial lesion; (2) superficially invasive squamous cell carcinoma; and (3) frankly invasive squamous cell carcinoma. Neoplasia incidence risk, neoplasia incidence rate, and cumulative probability of progression to neoplasia according to the Kaplan-Meier method were estimated. Log-rank test was used to compare the progression-free survival curves by age at diagnosis of VLS. The mean age at diagnosis of VLS was 60 (median = 60; range = 8-91) years. The mean length of follow-up was 52 (median = 21; range = 1-331) months. The following 34 patients developed a neoplasia: 8 VIN (4 dVIN, 4 high-grade squamous intraepithelial lesions), 6 keratinizing superficially invasive squamous cell carcinoma (5 with adjacent dVIN), and 20 keratinizing invasive squamous cell carcinoma (1 with adjacent dVIN). The neoplasia incidence risk was 3.5%. The neoplasia incidence rate was 8.1 per 1,000 person-years. The cumulative probability of progression to neoplasia increased from 1.2% at 24 months to 36.8% at 300 months. The median progression-free survival was significantly shorter in older women (≥70 years) when compared with that in younger women (p = .003). Vulvar lichen sclerosus has a nonnegligible risk of neoplastic transformation and requires a careful and lifelong follow-up in all patients, particularly in elderly women. Early clinical and histological detection of preinvasive lesions is essential to reduce the risk of vulvar cancer.
Vulvar cancer: a review fo dermatologists
Wiener Medizinische Wochenschrift
Vulvar malignancies are important tumors of the female reproductive system. They represent a serious health issue with an incidence between 2 and 7 per 100,000 and year. We provide a review about most important cancer entities, i.e., melanoma, squamous cell carcinoma, basal cell carcinoma, neuroendocrine cancer, and skin adnexal malignancies. Squamous cell carcinoma is the most common vulvar malignancy that can develop from vulvar intraepithelial neoplasia or de novo. Basal cell carcinoma represents only 2 % of all vulvar cancers. Melanoma of the vulva exists in two major types—superficial spreading and acral lentiginous. A special feature is the occurrence of multiple vulvar melanomas. Of the adnexal cancer types Paget’s disease and carcinoma are seen more frequently than other adnexal malignancies. The dermatologist should be aware of this problem, since he might be the first to be consulted by patients for vulvar disease. Treatment should be interdisciplinary in close association...
Vulvar Paget's disease--a case report
Collegium antropologicum, 2010
Vulvar Morbus Paget (MP) represents a rare intraepithelial adenocarcinoma. It accounts for less than 1% of all vulvar neoplasia and usually appears in postmenopausal women. Histologically it is analogous to Paget's disease of the breast. The most common clinical symptom is pruritus. The lesion appears as an erythematous or as an eczematous lesion with islands of hyperkeratosis. Occasionally, single anaplastic Paget's cells can be found on the vulvar smears which make cytological diagnosis of the disease possible. However, the disease can be diagnosed only by biopsy. We present a case of 49-year old woman with vulvar symptoms of pruritus, who had liver and kidney transplantation two years ago. During the standard gynecological examination the vulvar smear was taken for cytological evaluation. The smear was scanty, with inflammatory background, overloaded with squamae. There were two types of cells: dysplastic squamous cells from lower layer of the epithelium and the single, a...