Vulvar Cancer Facts

What is Vulvar Cancer?

Vulvar cancer develops when cells in the vulva begin growing uncontrollably. Normally, cells grow, divide, and die in an organized way. Cancer develops when changes in a cell's DNA allow abnormal cells to continue growing and potentially form a tumor.

The vulva includes several external structures of the female reproductive system:

• Labia majora: The outer folds of skin surrounding the genital area.

• Labia minora: The inner folds of skin surrounding the vaginal and urethral openings.

• Clitoris: A sensitive organ involved in sexual function.

• Vaginal opening: The external entrance to the vagina.

• Perineum: The area between the vaginal opening and anus.

Vulvar cancer most commonly develops on the labia majora or labia minora, although it can occur anywhere on the vulva.

What are the different types of vulvar cancer?

The type of vulvar cancer is determined by the cells in which the cancer begins.

Squamous cell carcinoma

Squamous cell carcinoma accounts for approximately 90% of vulvar cancers. It develops in the squamous cells that make up much of the vulvar skin.

There are two major pathways by which vulvar squamous cell carcinoma can develop.

HPV-associated vulvar cancer

Some vulvar cancers are caused by persistent infection with high-risk types of human papillomavirus (HPV), particularly HPV-16.

HPV is a common virus transmitted through intimate skin-to-skin contact. Most HPV infections clear naturally, but persistent infection with certain high-risk types can cause abnormal cellular changes that may eventually develop into cancer.

HPV-independent vulvar cancer

Other vulvar cancers develop without HPV infection. These cancers are more common in older adults and may be associated with chronic inflammatory skin conditions, particularly lichen sclerosus.

These cancers can develop through a different precancerous pathway.

Vulvar melanoma

Vulvar melanoma develops from melanocytes, the cells that produce pigment in the skin.

It is much less common than squamous cell carcinoma and behaves differently. Treatment is often guided by principles used for melanoma at other body sites, adapted to the vulvar location.

Basal cell carcinoma

Basal cell carcinoma is a rare type of vulvar cancer that begins in basal cells in the skin. It generally grows slowly and is less likely to spread than many other vulvar cancers.

Other rare types

Other uncommon vulvar malignancies include adenocarcinomas, certain glandular cancers, and sarcomas.

Paget disease of the vulva is a rare condition involving abnormal glandular-type cells in the vulvar skin. It may be noninvasive or associated with an underlying invasive cancer.

What is vulvar intraepithelial neoplasia?

Vulvar intraepithelial neoplasia, commonly called VIN, describes abnormal cells in the surface layer of vulvar skin that have not invaded deeper tissue.

VIN is not invasive vulvar cancer, but some types can progress to cancer if left untreated.

Two important categories are:

• Vulvar high-grade squamous intraepithelial lesion (VHSIL): Usually associated with high-risk HPV infection.

• Differentiated vulvar intraepithelial neoplasia (dVIN): Usually HPV-independent and often associated with lichen sclerosus. It has a greater tendency to progress to invasive cancer.

Doctors may recommend treatment or close monitoring depending on the type of lesion and individual risk factors.

Can vulvar cancer spread?

Yes. If cancer cells invade deeper tissues, they may spread to nearby structures or lymph nodes.

Vulvar cancer most commonly spreads first to lymph nodes in the groin. More advanced cancer may involve the vagina, urethra, anus, or distant organs.