The four stages of vulvar cancer correspond with how far the cancer has spread. Stage 4 vulvar cancer is the most advanced stage. It means the cancer has spread to the upper part of the urethra, vagina, or other parts of the body.

Vulvar cancer is a rare cancer that starts in the vulvar tissues. The American Cancer Society estimates that 6,470 vulvar cancers will be diagnosed in the United States in 2023, and about 1,670 people will die of this cancer.

The vulva includes the:

  • inner and outer vaginal lips (labia minora and labia majora)
  • clitoris
  • vaginal opening and its glands
  • mons pubis (the tissue that covers the public bone)

Most commonly, vulvar cancer is found in the outer vaginal lips. It’s usually a slow-growing cancer, starting with abnormal cells called vulvar intraepithelial neoplasia (VIN). Over time, VIN can become vulvar cancer, so early diagnosis and treatment is important.

In this article, we look at how doctors classify stage 4 vulvar cancer, along with treatment and survival rates.

Illustration showing the anatomy of the vulvaShare on Pinterest
Anatomy of the vulva. Medical illustration by Wenzdai Figueroa

Cancer staging determines how far the cancer has spread, and helps guide treatment decisions and prognosis.

Stage 4 vulvar cancer is the most advanced stage of vulvar cancer. There’s no stage 5. Doctors stage vulvar cancer through surgery, imaging scans, and other tests.

In stage four vulvar cancer, the cancer has spread to the upper part of the vagina or the upper urethra, or to another area of the body. There are two substages of stage four vulvar cancer: stage 4A and 4B.

StageDescription
4AThe cancer is in the vulva or the perineum or both, and may be growing into the anus, lower vagina, or lower urethra. The cancer may have spread to nearby lymph nodes and has attached to the underlying tissue or caused an ulcer or ulcers to form on one or more lymph nodes. The cancer has not yet spread to distant sites.

or

The cancer has spread to the bladder, rectum, pelvic bone, or upper part of the urethra or vagina. It may or may not have spread to nearby lymph nodes. It has not yet spread to distant sites.

4BThe cancer has spread to distant lymph nodes or organs like the lungs or bone. The cancer may or may not have spread to nearby organs or lymph nodes.

Vulvar cancer is usually a slow-growing cancer that often starts on the outer vaginal lips. If not diagnosed and treated, it can spread to other tissues on the vulva, and then to areas like the vagina, anus, urethra, lymph nodes, and other parts of the body.

Because it’s typically a slow-growing cancer, regular pelvic exams can help with early identification and treatment of abnormal tissues, and reduce the risk of spread.

Treatment for stage 4 vulvar cancer depends on whether it’s stage 4A or 4B.

Stage 4A treatment

In stage 4A, treatment can include:

  • surgery (radical vulvectomy or pelvic exenteration)
  • surgery and radiation
  • radiation or chemotherapy and radiation, then surgery
  • radiation, with or without chemotherapy

Your treatment team will discuss your options with you, and depending on your overall health and the specifics of your cancer and where it has spread, your options may vary. T

reatment may not be able to cure the cancer at this stage, but it can help ease symptoms, reduce tumor size, and improve quality of life.

Stage 4B treatment

According to the National Cancer Institute (NIH), there’s no standard treatment for stage 4B vulvar cancer. Doctors may treat cancer that has spread to other areas of the body with chemotherapy or immunotherapy. Palliative treatments may also be used to help ease symptoms.

Treatment guidelines from the National Comprehensive Cancer Network (NCCN) for stage 4B vulvar cancer recommend External Beam Radiation Therapy (EBRT) to relieve symptoms and control the cancer’s further spread.

Doctors may also use systemic therapies or Best Supportive Care (BSC) to manage cancer-related symptoms. Per the NCCN, the preferred first-line systemic therapies for stage 4B vulvar cancer include:

  • cisplatin/paclitaxel/bevacizumabb
  • cisplatin/paclitaxel
  • carboplatin/paclitaxel
  • carboplatin/paclitaxel/bevacizumab

Other recommended medications by the NCCN for stage 4B vulvar cancer include:

  • cisplatin
  • carboplatin

Survival rates vary based on the stage of vulvar cancer and the amount of spread. For stage 4 vulvar cancer, the 5-year relative survival rate is 22.9%.

A relative survival rate is an estimate of how long someone with a specific condition may live after their diagnosis compared with someone without the condition. For example, a 5-year relative survival rate of 22.9% means that someone with that condition is 29.9% as likely to live for 5 years as someone without the condition.

  • Localized (only in the vulva): 86.2%
  • Regional (spread only to lymph nodes in the vulvar area): 48.4%
  • Distant (spread to other areas of the body): 22.9%
  • Overall (combined survival rate): 70.3%

It’s important to remember that statistics are a good estimate, but each person is different, and these are not absolutes.

Factors influencing survival rates

Lymph node involvement has been found to influence survival. This means that whether there’s cancer in the lymph nodes — and how many — can impact survival rates.

The stage at which a person is diagnosed with vulvar cancer also affects survival rates. Being diagnosed at a later stage is associated with a lower rate of survival.

Other factors that may impact survival include your age, the type and size of the tumor, and your overall health.

Vulvar cancer is a slow-growing cancer that begins in the vulvar tissues. Like other cancers, it’s broken down into stages, which provide an idea of how far the cancer has spread and help to guide treatment.

Stage 4 vulvar cancer is the most advanced stage, and may involve vulvar cancer that has spread to other areas of the body.

There are multiple treatments available for stage 4 vulvar cancer, including surgery, chemotherapy, and radiation. Your treatment team will discuss with you the most appropriate treatment for your specific medical situation.