Vulvar cancer is staged based on the extent it has spread. During the early stage, the cancer remains in the vulva. In its most advanced stage, the cancer has spread to distant organs and tissue.

After diagnosis, doctors use staging to determine the options appropriate for treating vulvar cancer. These four stages differ based on how far the tumor has spread.

Two staging systems are used to help doctors account for the size and extent of the tumor. They also examine whether the cancer has spread to nearby lymph nodes or distant organs and tissues.

If you or a loved one have been diagnosed with vulvar carcinoma, you’re not alone. Understanding how vulvar carcinoma is staged may help you better identify signs of cancer and communicate concerns with a healthcare professional. It can also help you better understand the different types of treatment available to you.

Vulvar cancer stages range from 1 to 4. Stage 1 represents the earliest stage of the disease, and Stage 4 represents the most advanced stage. The staging process helps doctors determine whether and to what extent the cancer has spread.

Some stages include substages to better define where the cancer has spread. This categorization is vital when choosing an approach to potentially reduce the size and spread of the tumor. Doctors can also use it to predict the outlook for people living with vulvar carcinoma.

Doctors use one of two staging systems to stage VC:

  • International Federation of Gynecology and Obstetrics system (FIGO)
  • American Joint Committee on Cancer (AJCC) TNM staging system

Both the FIGO system and the AJCC TNM system stage vulvar cancer using the TNM staging system. This stands for:

  • T (Tumor): This denotes the size of the tumor and the extent to which it has grown into nearby structures and organs, such as the bladder or rectum.
  • N (Nodes): This denotes the number of nearby lymph nodes to which the cancer has spread and whether it has spread beyond them.
  • M (Metastases): This denotes whether the cancer has spread or “metastasized” to distant lymph nodes or organs.

Doctors assign a number after each letter to provide more information about each factor. Generally, higher numbers indicate that the cancer is more advanced.

Once a doctor has assigned numbers to each of the TNM categories, they will use this information to assign an overall cancer stage. These stages are outlined below.

Stage 1 VC is the earliest stage of the disease, during which the tumor is confined to the vulva.

Stage 1 VC is further divisible into two substages, 1A and 1B:

  • Stage 1A: The tumor (T) measures 2 centimeters (cm) or less and has spread 1 millimeter (mm) or less into the vulvar tissue. The cancer has not spread to the lymph nodes (N0) and has not metastasized (M0) to distant parts of the body. The TNM classification for this is “T1A, N0, M0.”
  • Stage 1B: The tumor (T) is larger than 2 cm or has spread more than 1 mm into the vulvar tissue. The cancer has not spread to the lymph nodes (N0) and has not metastasized (M0) to distant parts of the body. The TNM classification for this stage is “T1B, N0, M0.”

Stage 2 VC is more advanced than Stage 1. In this stage, the tumor may be any size, and is growing into one of the following parts of the body:

  • anus
  • lower one-third of the vagina
  • lower one-third of the urethra, the tube that drains urine from the bladder

The cancer has not spread to the lymph nodes (N0) and has not metastasized to distant sites (M0).

The TNM classification for this stage is “T2, N0, M0.”

Stage 3 VC is divisible into three subcategories, depending on the extent to which the cancer has spread. In all cases, the tumor (T) may be any size and categorized as one of the following:

  • T1: The tumor is in the vulva, in the tissue between the vulva and anus called the “perineum,” or in both areas.
  • T2: The tumor is in the vulva and/or perineum. It’s also growing into the lower vagina, lower urethra, or anus.

Below is a more detailed description of the Stage 3 VC subcategories.

Stage 3A

The cancer has spread to nearby lymph nodes (N) in either of the following ways:

  • The cancer has spread to a single nearby lymph node, with the area of spread measuring 5 mm or more (N1).
  • The cancer has spread to one or two nearby lymph nodes, with both areas of spread measuring less than 5 mm (N1).

The cancer has not metastasized (M0) to distant sites.

Stage 3B

The cancer has spread to nearby lymph nodes (N) in either of the following ways:

  • The cancer has spread to three or more nearby lymph nodes, with all areas of cancer spread measuring less than 5 mm (N2a).
  • The cancer has spread to two or more lymph nodes, with each area of spread measuring 5 mm or more (N2b).

The cancer has not metastasized (M0) to distant sites.

Stage 3C

The defining feature of Stage 3C in vulvar cancer is extracapsular spread.

This occurs when the cancer has spread and begins to grow through the outer covering of at least one of the nearby lymph nodes (N2c).

The cancer has not metastasized (M0) to distant sites.

Stage 4 vulvar cancer is the most advanced stage. It’s divisible into two subcategories depending on the extent to which the cancer has spread.

Below is a more detailed description of the two Stage 4 subcategories.

Stage 4A

The cancer has spread to one of the nearby lymph nodes, where it has become stuck to the underlying tissue or caused an ulcer to form on the lymph node (N3).

Stage 4A may also be assigned if the spread beyond nearby tissues to the upper part of the vagina or urethra (T3). The increased size may also cause it to spread to one of the following:

  • bladder
  • rectum
  • pelvic bone

With T3 categorization, the tumor may or may not spread to nearby lymph nodes.

The cancer has not spread to distant sites (M0).

Stage 4B

The defining feature of Stage 4B vulvar cancer is the tumor spreading to distant lymph nodes, or organs and tissues (M1).

Categorization based on the staging systems can differ from person to person in this stage. This is because the tumor will vary in size and the extent to which it has spread to nearby lymph nodes and organs.

According to a 2023 article, vulvar cancer does not always cause symptoms, especially during the early stages of the disease. However, some people living with this condition may experience:

  • vulvar pain or itching
  • an ulcer on the vulva
  • a lump or bump on the vulva

Vulvar lumps and bumps may be red, pink, or white. They may have a rough, thickened, or warty surface. Some may appear as cauliflower-like warty growths.

Other potential symptoms of vulvar cancer include:

  • vulvar pain, burning, or itching
  • thickening of the skin of the vulva
  • an open sore, especially one that lasts a month or more
  • an area of darkened, lightened, or reddish skin on the vulva
  • unusual bleeding or discharge that is not related to menstruation

As 2024 research explains, surgery is the standard treatment for vulvar cancer, especially in its early stages. Depending on the extent of the disease, doctors may also recommend other cancer treatments, such as:

  • Radiation therapy: Radiation therapy uses high-energy particles or waves to destroy cancer cells. It may be recommended after surgery to help destroy any remaining cancer cells. Doctors may also recommend it as an alternative for people who are not well enough to undergo surgery.
  • Chemotherapy: Chemotherapy drugs are medications that help destroy cancer cells or prevent their growth. The American Cancer Society (ACS) reports that there are no standard chemotherapy treatment plans for vulvar cancer.
  • Chemoradiation therapy: Chemoradiation therapy refers to a combination of chemotherapy and radiation therapy. Doctors may recommend this treatment for more advanced vulvar cancer.

According to the National Cancer Institute, the 5-year relative survival rate (RSR) for all types of vulvar cancer combined is approximately 70%. This survival rate measures the number of people who are still alive 5 years after their initial diagnosis of vulvar cancer.

However, survival rates differ according to the extent of the disease at the time of diagnosis. The survival data for vulvar cancer indicates how far the cancer has spread in the following ways:

  • Localized or “stage 1” cancer: This is cancer that has not spread beyond the area where it first developed. The 5-year RSR for localized VC is nearly 86%.
  • Regional cancer: This is cancer that has spread to nearby lymph nodes or other nearby tissues: The 5-year RSR for regional VC is about 47%.
  • Advanced cancer: This is cancer that has spread to distant organs and tissues. The 5-year RSR for advanced VC is about 22%.

As a 2023 article explains, vulvar cancer has a high rate of recurrence, so regular check-ups are necessary to help ensure the cancer has not come back.

If you have had vulvar cancer, you should receive a checkup every 3 months for the first 2 years after your treatment. Following those 2 years, checkup frequency is recommended to change to every 6 months for the following 3 years.

After this, you should have a checkup at least once a year.

Vulvar cancer is a rare type of cancer that affects the vulva. Part of the diagnostic workup involves staging the cancer to determine the extent to which it has spread.

Doctors use one of two staging systems to stage vulvar carcinoma: the FIGO system and the AJCC TNM system. They use information from these staging systems to assign an overall vulvar cancer stage ranging from 1 to 4.

Stage 1 represents the earliest stage, and Stage 4 is the most advanced. This helps doctors develop an appropriate treatment plan and can also give insights into individual outlooks.

Surgery is the main treatment for vulvar cancer. However, doctors may also recommend radiation therapy, chemotherapy, or a combination of both.

Early-stage vulvar cancer tends to have a better treatment response and a more favorable outlook, highlighting the importance of early diagnosis and treatment.