Vulvar Cancer: Overview

Neha Vapiwala, MD and Eric T. Shinohara, MD, MSCI
The Abramson Cancer Center of the University of Pennsylvania
Last Modified: February 23, 2008

What is vulvar cancer?

It is an abnormal growth of malignant cells (neoplasm, tumor) in the vulva. The vulva is defined as the female external genitalia, and includes the labia majora, labia minora, clitoris, and vestibule, or entryway, of the vagina.

About 70% of vulvar cancers involve the labia, (mainly the labia majora), 15% - 20% involve the clitoris, and another 15% - 20% involve the perineum, which is the area of sensitive skin located between the vagina and the anus. In about 5% of cases, the cancer is present at more than one site.

What are the different types of vulvar cancer?

The vulva is essentially epithelial skin, and so the main tumor types that affect this area are skin-related cancers.

The majority of vulvar cancers are squamous cell carcinomas, which typically develop at the edges of the labia majora/ minora or the vagina. As with vaginal squamous cell carcinomas, vulvar squamous cell cancers are slow-growing and usually develop from "precancerous", pre-invasive areas called vulvar intraepithelial neoplasia (VIN).

Melanoma is the second most common type and represents about 5% - 10% of vulvar cases; roughly half involve the labia majora.

The most common skin-cancer in sun-exposed areas is basal cell carcinoma, and as expected, this type rarely occurs on the vulva.

Adenocarcinomas of the vulva are also rare, but can develop from glands such as the Bartholin's glands at the vaginal opening.

How common is vulvar cancer?

Vulvar cancer is a relatively rare diagnosis, representing about 5% of all gynecologic cancers, and only about 1% of all female cancers in general. There are about 3,500 new cases reported annually in the US and approximately 900 death a year attributed to this disease. The incidence of vulvar cancers has remained stable over the past two decades but the rates of precancerous lesions has more than doubled over the same period of time. The cause for the growing number of cases is not well-understood.

Who gets vulvar cancer?

Vulvar cancer most commonly occurs in postmenopausal women. The average age at diagnosis is 65, but studies have suggested that vulvar cancers may be becoming more common in women in their 50’s.

What are the risk factors for vulvar cancer?

In addition to older age, vulvar cancer has been associated with a history of:

  • infection with high-risk HPV types, (ie: HPV 16,18,33)
  • multiple sexual partners/ sexually transmitted diseases
  • cervical cancer
  • immunodeficiency
  • presence of chronic vaginal and vulvar irritation
  • smoking

What are the symptoms of vulvar cancer?

The classic symptom is vulvar itching (pruritus), reported in almost 90% of the women with vulvar cancer. There can also be associated pain, bleeding, vaginal discharge, and/or painful urination (dysuria).

Also, women often develop a visible vulvar mass: the squamous cell subtype can look like elevated white, pink or red bumps, while vulvar melanoma characteristically presents as a colored, ulcerated growth. There can be portions of the tumor that look sore and scaly, or cauliflower-like (similar to HPV-related warts).

How is vulvar cancer diagnosed?

First and foremost, a thorough gynecological examination should be performed using a colposcope (special magnifying instrument) for better visualization. Any suspicious areas should be tested by applying a dilute solution of acetic acid to the region; abnormal areas typically turn white, making them easier to identify. Also, any abnormal-appearing area should be sampled along with surrounding normal tissue using a thick wedge-shaped biopsy (usually under local anesthesia). If the area is small, it should be entirely removed in the process of the biopsy (so-called excisional biopsy). Only rarely (5% of the time) are multiple masses seen at the time of diagnosis (meaning that the vulvar cancer has shown up in two separate places). However, a second cancer (most commonly cervical cancer) can be found in up to 22% of women with vulvar cancer. Hence, a careful exam of all of the skin in the vaginal and groin area, as well as a gynecological exam should be performed. A Pap smear should be performed and additional smears taken from the vagina and vulva should be collected and tested. Lymph node sampling is required in all people, except for those with the earliest stage of disease. It appears that a sentinel lymph node biopsy (where a dye or a radioactive compound is used to determine which lymph nodes drain the area of the lesion) may be adequate for lymph node sampling.

Chest x-ray and CT scan of the abdomen/pelvis can be done to look for disease spread to lymph nodes and/or distant organs. If spread to bladder or rectum is suspected, endoscopy (cystoscopy and proctoscopy, respectively) should be performed.

Once it is diagnosed, how is vulvar cancer staged?

Vulvar cancer can spread by direct extension, meaning that they can grow into adjacent areas such as the vagina and anus. Even in early disease, spread to lymph nodes can occur. However, spread to other organs is rare until late in the course of the disease. Unlike vaginal cancer, which is typically clinically staged, the International Federation of Gynecology and Obstetrics (FIGO) uses a surgical staging system for vulvar cancer. This means that the stage of the cancer is not actually determined until after surgery is performed and the specimen is examined by the pathologist.

Like vaginal cancer, vulvar cancer has five main FIGO stages (0, I, II, III, and IV). They are:

  • Stage 0 - Vulvar intraepithelial neoplasia
  • Stage I - cancer is limited to the vulva and perineum, and measures < 2 cm in size
  • Stage II - cancer is limited to the vulva and perineum, but tumor is > 2 cm in size
  • Stage III - cancer spread to vagina, urethra, anus, and/or the lymph nodes in the groin
  • Stage IV - cancer spread to bladder, bowel, pelvic bone, pelvic lymph nodes, and/or other parts of the body

How is vulvar cancer treated?

Surgery, radiation therapy and chemotherapy are the main treatment options, and are typically used in various combinations. As with many cancers, the optimal treatment depends on the disease stage and patient factors such as age and other medical conditions.

Treatment options by stage are as follows:

Stage 0

  • Wide local excision, laser surgery, or a combination of both
  • Skinning vulvectomy
  • Chemotherapy ointment

Stage I

  • Wide local excision
  • Radical local excision with removal of all nearby groin/ upper thigh lymph nodes
  • Radical vulvectomy and removal of nearby groin lymph nodes (and sometimes lymph nodes on opposite side of the body)
  • Radiation therapy alone (in selected patients)

Stage II

  • Radical vulvectomy and removal of groin lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis if lymph nodes are positive for cancer
  • Radiation therapy alone (in selected patients)

Stage III

  • Radical vulvectomy and removal of groin/ upper thigh lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis and groin if lymph nodes are positive for cancer or if the primary vulvar tumor is very large
  • Radiation therapy and chemotherapy, followed by radical vulvectomy and removal of lymph node removal of lymph nodes on both sides of the body.
  • Radiation therapy (in selected patients) with or without chemotherapy.

Stage IV

  • Pelvic exenteration, which entails radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread), as well as the uterus, cervix, and vagina
  • Radical vulvectomy followed by radiation therapy
  • Radiation therapy followed by radical vulvectomy
  • Radiation therapy (in selected patients) with or without chemotherapy, and possibly following surgery

After treatment, following up with your physician is essential. It is recommended that after treatment, a gynecologic examination should be performed at least twice a year with careful inspection of the skin and inguinal nodes. If any abnormalities are seen a colposcopy and biopsy should be performed. Up to 10% of recurrences can occur after 5 years underscoring the importance of long follow up.

Local recurrence (near where the cancer was originally found) is the most commonly place where cancers can recur. These can often be successfully treated with surgery. If the disease has spread to other organs (distant disease) it can be treated with chemotherapy. The most frequently used chemotherapies include cisplatin, methotrexate, bleomycin and mitomycin C. However, the response rate to these chemotherapies is low and the length of response tends to be brief. There is ongoing research examining the use of new biological agents, such as erlotinib, which appear to have good early results.

What is the prognosis?

For stage I disease the 5 year survival is approximately 77%. For stage II and III disease, five- year survival is about 55% and 31% respectively. Survival at five years for stage IV disease is rare.