AssessmentsVulvar cancer: Clinical practice
USMLE® Step 1 style questions USMLE
USMLE® Step 2 style questions USMLE
A 50-year-old woman comes to her gynecologist's office because of itching and pain in her genital area for the past few months. Her past medical history is significant for hypertension, insulin-controlled diabetes mellitus, and multiple abnormal Pap smears. She has smoked one pack of cigarettes daily for the past 35 years. She has been taking oral contraceptives since her teen years. Family history is negative for gynecologic disease. Pelvic examination shows multiple excoriations and an ulcerated mass in the vulva. Vulvar punch biopsy shows vulvar intraepithelial neoplasia III (VIN III). Which of the following human papilloma virus (HPV) strains are most likely associated with this patient's disease?
Over 90% of vulvar cancers are squamous cell carcinomas, which develop from the squamous epithelium of the vulva.
The other 10% of vulvar cancers are melanomas, which develop from melanocytes, and vulvar adenocarcinomas, which develop from glandular cells, like those that make up Bartholin’s glands on either side of the vaginal opening.
A particular type of adenocarcinoma is Paget’s disease of the vulva - which is a type of cancer that usually develops in the ducts of the breast, but can rarely develop in other locations, such as the vulva.
And just like cervical cancer, vulvar cancer starts out as vulvar intraepithelial neoplasia, and it may take many years for it to even progress to cancer.
Other risk factors for squamous cell carcinomas include smoking, immunosuppressive conditions like an HIV infection, and lichen sclerosus, which is a skin disorder than frequently affects the genitals, making the skin become very thin and white, like cigarette paper.
For the melanoma type of vulvar cancers, risk factors include a family history of melanoma, or a personal history of melanoma or dysplastic nevi, which are atypical moles, in other locations on the body.
Additionally, individuals with cervical or vaginal cancer have a higher risk of vulvar cancer, probably because they can all be caused by high risk HPV strains.
Symptoms vary depending on histologic subtype, but at the end of the day, there’s always a vulvar lesion, and pruritus - the fancy word for itching.
Sometimes there may also be associated pain, bleeding or discharge. Squamous cell carcinomas usually look like a red, pink or white bump, or it may look like a wart, or an open sore that doesn’t heal.
Vulvar melanomas, on the other hand, are usually black or dark brown - and sometimes they may start in a mole, in which case the ABCDE rule can be used to distinguish the cancer from the mole. A is for asymmetry, which means one half of the mole looks different than the other, B is for border irregularity, C is for color, meaning there are various colors within the same lesion, D is for a diameter wider than 6 millimeters, and E is for evolving - meaning the mole changes in size, shape or color over time.
Adenocarcinomas that develop from Bartholin’s glands, present like a distinct lump on either side of the opening of the vagina. Finally, Paget’s disease of the vulva presents as a red, scaly, sore lesion.
To diagnose vulvar cancer, a biopsy is done. If subclinical vulvar lesions are suspected, a vulvoscopy may also be done - which is when a colposcope is used to get a zoomed-in view of the vulva, and any suspicious looking areas are biopsied.
The biopsy can be an excisional biopsy, when the lesion is small and it’s removed entirely, along with a little bit of tissue around it, or a punch biopsy, when just a sample is taken.
The biopsy confirms the malignant character of the lesion, and it also differentiates between the histologic subtypes, and this guides further treatment.
Additionally, for squamous cell carcinoma, a biopsy may identify precancerous lesions - which may be either low grade squamous intraepithelial lesion, or LSIL, or high grade squamous intraepithelial lesion, or HSIL, depending on how atypical the cells look.
LSIL vulvar intraepithelial neoplasia is equivalent to warts, and they can be treated with local cytodestructive therapies like podophyllotoxin, which are applied by the individual, or acid and acid, which are applied by a clinician.
Immunotherapeutic agents like imiquimod creams can also be given, and they’re also applied by the individual. Alternatively, the lesion can be removed through ablative procedures, like cryoablation, laser ablation, or electrocautery, or excisional procedures.