Vulval disorders

The vulva is an encompassing term to describe all the parts of the external genitalia in women. It includes the vestibule, labia majora, labia minora and clitoris.

The vestibule is a smooth surface that begins below the clitoris and contains both the urethral opening (anteriorly) and the vaginal orifice (posteriorly). Vulval disorders refers to conditions affecting the labia, clitoris or vestibule.

In this CCC we will focus on vulval cancer and genital warts. Other vulval disorders that have been discussed separately include Bartholin’s cysts, and lichen sclerosus.

VULVAL CANCER

Vulval cancer is a relatively rare gynaecological cancer with only 1200 cases diagnosed annually in the UK. Around 80% are squamous cell carcinomas but they may also malignant melanomas sometimes.

⚠️ Risk factors

😷 Presentation

Vulval cancer may be an incidental finding in older women, for example during catheterisation.

Signs and symptoms include:

• Vulval lump
• Ulceration
• Bleeding

• Pain and dyspareunia
• Itching and irritation
• Inguinal lymphadenopathy

It most commonly affects the labia majora which gives the appearance of:

1. Irregular mass
2. Fungating lesion - ulcerated and necrotic and has an odorous smell.
3. Ulceration
4. Bleeding

🔍 Investigations

🥇 Physical examination can raise suspicion if there is a lump or if it is ulcerated.

🏆 Biopsy can confirm diagnosis.

CT abdomen pelvis is used for staging the disease.

🔢 Staging

The International Federation of Gynaecology and Obstetrics (FIGO) classification system is used in staging vulval cancer.

1. Stage I - tumour confined to vulva, <2cm in dimension.
1. Stage Ia - stromal invasion <1mm
2. Stage Ib - stromal invasion >1mm
2. Stage II - tumour of any size with extension to the lower 1/3rd of the urethra, lower 1/3rd of the vagina and lower 1/3rd of the anus with negative nodes.
3. Stage III - tumour of any size with extension to upper part of the adjacent perineal structures or with any number of non-fixed, non-ulcerated lymph nodes.
1. Stage IIIa - Tumor of any size with disease extension to upper two-thirds of the urethra, upper two-thirds of the vagina, bladder mucosa, rectal mucosa, or regional lymph node metastases ≤ 5 mm
2. Stage IIIb - Regional lymph node metastases > 5 mm
3. Stage IIIc - Regional lymph node metastases with extracapsular spread
4. Stage IV - tumour of any size fixed to bone or fixed, ulcerated lymph node metastases, or distant metastases.
1. Stage IVa - Disease fixed to the pelvic bone or fixed or ulcerated regional lymph node metastases.
2. Stage IVb - Distant metastases

🧰 Management

VULVAL INTRAEPITHELIAL NEOPLASIA (VIN)

VIN is a premalignant condition that affects the squamous epithelium of the skin. It precedes vulval cancer and can be considered stag 0 vulval cancer.

Grading for VIN used to be 1-3, however, it has since been renamed:

1. Low grade squamous intraepithelial lesion (LSIL) - replaces VIN 1.
2. High grade squamous intraepithelial lesion (HSIL) - replaces VIN 2 & 3. It is the most common type of VIN and is associated with HPV infection that typically occurs in women aged 35-50 years old.
3. Differentiated VIN (dVIN) - associated with lichen sclerosus and typically occurs in older women aged 50-60 years old.

🔍 It needs to be biopsied to be diagnosed

😷 Presentation

• Asymptomatic
• Itching
• Pain
• Changes to vulval skin
• Dyspareunia

🧰 Management

• 🥇 Watch and wait
• Wide local excision
• Imiquimod cream - usually used to treat genital warts but can be used for VIN.
• Laser ablation

GENITAL WARTS

Anogenital warts, also known as condylomata acuminata, are benign, proliferative growths that are found on the genital, perianal and anal regions due to infection by HPV. The most common sites are the vaginal introitus, under the foreskin of an uncircumcised penis, and on the shaft of a circumcised penis. However, they may occur less commonly at the urethral meatus, vagina, cervix and anal canal. Recurrence is common even after treatment.

Pathophysiology

They are most commonly caused by HPV 6 & 11. They enter the skin and infect keratinocytes to form koilocytes - these are epithelial cells that have undergone structural changes after infection from HPV. The incubation period is shorter in women (~3 months), as compared to men (11 months).

It is transmitted via direct contact with a person with HPV or through genital secretions of an infected individual. Fomite transmission is also possible as with auto-inoculation from one site to another.

😷 Presentation

Individuals are usually asymptomatic.

There may be one or multiple warts present in areas of high friction or areas traumatised during sexual intercourse. They present as cauliflower-like growths.

Depending on the the type of skin they are on, they can be either keratinised or not:

• Non-hairy skin - soft and non-keratinised.
• Dry hairy skin - firm and keratinised.

They are sometimes itchy.

• Bleeding - may occur due to local trauma.
• Urinary symptoms - may occur if the lesion is in the distal urethra and near the meatus.

🔍 Investigations

It is a clinical diagnosis.

🧰 Management

It is essential to screen for co-existing STIs, especially in individuals <25 years old. This can be done by:

1. Vaginal speculum examination
2. Proctoscopy - if there is a history of receptive anal sex.