Lichen sclerosis

• Occurs at all age groups but is more common after the menopause
• Possible autoimmune
• Leads to atrophy, fibrosis and scarring
• Skin becomes pale grey and parchment like
• The labia become atrophied
• Introitus becomes narrowed
• It is not recognised as being precancerous but a greater risk of subsequent carcinoma may occur in 1-4% of cases

Histological features

• Atrophy of the epidermis with disappearance of the rete pegs
• Hydropic degeneration of the basal cells
• Replacement of underlying dermis with dense collagenous fibrinous tissue
• Monoclonal bandlike lymphocytic appearance

Lichen Simplex Chronicus

• Non specific, secondary to rubbing the skin to relieve pruritus
• Presents as white vulvar plaques
• Results in thickened epidermis (showing increased mitotic activity), hyperkeratosis and variable leukocytic infiltrate of the dermis
• Not considered a cancer precursor but may be associated with carcinoma

Carcinoma of the Vulva

• Uncommon, represents 3% of all female genital cancers
• Mostly seen in women over 60
• 85% of malignancies are squamous cell carcinomas, the rest basal cell carcinomas, adenocarcinomas or melanomas
• Squamous carcinomas can be divided into 2 groups depending on aetiology and pathogenesis, (1) those associated with HPV and (2) those associated with lichen sclerosis

• Those associated with high risk HPV frequently coexist or are preceded by a precancerous stage called vulvar intraepithelial neoplasia (VIN)
• VIN is characterised by nuclear atypia, increased mitosis and loss of cell surface differentiation
• It is analogous to CIN
• VIN is frequently multicentric, 10-30% are associated with primary squamous neoplasm is the vagina or cervix
• VIN is associated with HPV 16 and 18
• Spontaneous regression of VIN has been reported in younger women
• Risk of progression is greater with age or immunosuppression

• The second group of squamous cell carcinoma is associated with squamous cell hyperplasia and lichen sclerosis
• Aetiology is unclear
• Leads to a form of VIN termed ‘undifferentiated’
• These tumours are associated with accumulation of p53 protein

• Once invasive cancer develops, metastatic spread is associated with the tumour size, depth of invasion and association with lymphatic vessel
• Inguinal, pelvic, iliac and periaortic vessels are commonly involved
• Metastases generally go to lung and liver
• Lesions less than 2cm have a 5 year survival rate of 60-80% following vulvectomy and lymphadenopathy
• Larger lesions with lymph node involvement have a 10% 5 year survival rate

Vagina

• Vaginal disorders are generally uncommon
• Infection is the commonest disease process

Vaginal Malignancy

Squamous cell carcinoma

• Primary carcinoma of the vagina is very rare, accounting for 1% of malignancies of the female genital tract
• 95% of these are squamous cell carcinomas and are associated with HPV
• The greatest risk factor is previous carcinoma of the cervix or vulva, around 1% of patients with an invasive cervical carcinoma develop a vaginal squamous carcinoma

Histology Squamous cell carcinoma

• Like cervical and vulval squamous cell carcinoma it is preceded by a premalignant stage, vaginal intraepithelial neoplasia (VAIN)
• Most often the tumour affects the upper posterior part of the vagina, at the junction with the endocervix

• Tumour presents with irregular spotting or vaginal discharge or is silent until urinary or bowel changes occur

Adenocarcinoma

• Adenocarcinoma of the vagina is rare but is associated with women whose mothers were treated with diethylstilbestrol whilst pregnant (to prevent abortion)
• 0.14% of DES exposed women develop adenocarcinoma
• Occurs in young women, age 15-20

Histology of Adenocarcinoma

• Adenocarcinoma tumours are more likely to occur on the anterior wall of the upper 1/3 of the vagina
• They are composed of vacuolated glycogen containing cells hence the reason it is referred to as clear cell carcinoma
• A possible precursor of the tumour is vaginal adenosis, where glandular columnar epithelium of Mullerian type appears under the squamous epithelium or replaces it