What Are the Major Causes of Vulvar Cancer? Risk Factors

Major risk factors for vulvar cancer include:

Human papillomavirus (HPV)

• One of the most frequent sexually transmitted infections. Vulvar cancer is frequently linked to HPV strains HPV-16 and HPV-18.
• Most women who are sexually active are exposed to the virus at some point. 
• Many HPV subtypes are asymptomatic and do not cause long-term health issues. 
• Other HPV subtypes can lay latent in the body for years before causing genital warts, vulvar cancer, or other malignancies on the genital tract, anus, or oral cavity.
• Vulvar cancer caused by HPV is more common in younger, sexually active women who smoke. Researchers think HPV causes inflammation in the body, which encourages cell proliferation, ultimately leading to precancerous cell alterations and/or vulvar cancer.

Vulvar dystrophies

• Vulvar malignancies associated with vulvar dystrophies are more common in elderly women.
• Squamous hyperplasia (causes itchy, reddish skin and elevated white regions) and lichen sclerosus (causes white patches of thinning skin) are two prevalent types of vulvar dystrophy.
• Women at risk of vulvar dystrophies may have gene mutations that influence tumor development. Mutations in the p53 tumor suppressor gene are thought to be the cause of vulvar dystrophies. When this gene fails to function correctly, vulvar cells may proliferate out of control or survive for longer than expected. 

Other risk factors for vulvar cancer include:

• Age (vulvar cancer is most common in women ages 70-80)
• Genital warts
• Sexual intercourse at a young age
• Multiple sex partners
• Being childless
• Long-term irritation or certain skin problems (lichen sclerosus and lichen simplex chronicus)
• History of vaginal or cervical cancer
• History of melanoma or several atypical moles
• Weak immune system
• Obesity
• Diabetes
• Smoking
• Radiotherapy to your pelvis in the past
• Exposure to diethylstilbestrol (DES) before birth while in the mother’s womb (in the 1950s, doctors prescribed DES to some pregnant women to prevent miscarriage)

Although these factors can increase the risk of developing vulvar cancer, they do not always cause the disease. Some women with one or more risk factors never develop cancer, whereas others develop cancer with no known risk factors. 

However, knowing risk factors for any cancer can help guide women to the appropriate actions, such as undergoing screening for the disease.

Vulvar cancer may not always cause symptoms in the early stages. Potential signs and symptoms of vulvar cancer include:

• Severe burning, especially when passing urine
• Pain during intercourse
• Constant itching
• Pain, soreness, or tenderness in the vulva area
• Changes in the color and appearance of the vulva
• Bleeding or discharge not related to menstruation
• Lumps or wart-like growths on the vulva
• Persistent or chronic sores on the vulva that do not heal
• Blood, pus, or discharge from a lesion or sore in the vulva area
• Hard or swollen lymph glands in the groin
• Foul-smelling or blood-stained vaginal discharge
• Abdominal pain 

Symptoms of vulvar cancer may resemble other conditions or medical problems. In order to confirm a diagnosis, doctors may rule out other conditions and recommend certain tests.

Doctors use a variety of systems to stage cancer. Ask your doctor to explain the stage of your cancer in a way that is easy to understand.

• Stage I:  Cancer may be found in the vulva region and is not present in nearby lymph nodes or other body regions
  • Stage IA: Tumor is no more than 2 cm wide. It has only grown 1 mm into the vulva tissue.
  • Stage IB: Tumor is more than 2 cm in diameter and/or has spread more than 1 mm beneath the skin's surface.
• Stage II: Tumor can be of any size and cancer has spread from the vulva to surrounding areas. Cancer may not have migrated to the lymph nodes or any other organs
• Stage III: Tumor has spread to the vulva, perineum, or both. It may have spread to the urethra, lower vaginal wall, or anus. It has not spread to other organs in the body.
  • Stage IIIA: Tumor has spread to at least 1 lymph node that is at least 5 mm in diameter or to 1-2 lymph nodes that are less than 5 mm in diameter.
  • Stage IIIB: Tumor has spread to 3 or more lymph nodes no larger than 5 mm in diameter or 2 or more lymph nodes larger than 5 mm in diameter.
  • Stage IIIC: Tumor has spread to surrounding lymph nodes, and at least 1 lymph node has malignancy developing through its outer covering (called extracapsular spread).
• Stage IV:
  • Stage IVA: Tumor has not spread to other organs in the body, and one of the following is true:
    • Tumor has spread to the vulva, perineum, or both. It may have spread to the urethra, lower vaginal wall, or anus. It has spread to neighboring lymph nodes, which have been encrusted with deeper tissues or have ulcerated.
    • Tumor has spread to the upper urethra, upper vagina, bladder, rectum, or pelvic bone from adjacent tissues. It may have migrated to adjacent lymph nodes.
  • Stage IVB: This is the most advanced stage. Cancer has spread to organs such as the lungs or bone, as well as lymph nodes located elsewhere in the body. Tumor may have spread to adjacent lymph nodes and organs.

Stage I treatment

• Treatment may include partial vulvectomy and inguinal lymph node dissection (removal of nearby groin lymph nodes).
• Instead of lymph node dissection, a sentinel lymph node biopsy can be performed. Radiation with chemotherapy is given if cancer is detected in the lymph nodes.
• A total radical vulvectomy with excision of the groin lymph nodes is performed for tumors that are bigger and more widespread.
• If a woman is unable to have surgery, her lymph nodes are not removed; instead, radiation treatment to the groin is administered.

Stage II treatment

• Treatment may include partial radical vulvectomy.
• Lymph nodes in the groin on both sides of the body are removed or sentinel node biopsies are done.
• After surgery, radiation therapy can also be given; if cancer cells are at or near the edges of the tissue, they are removed during surgery.
• Radiation with or without chemotherapy can be used as the main treatment for women who are ill or weak due to other medical problems.

Stage III treatment

• Treatment includes surgery to remove cancer (either a radical wide local excision or partial or complete radical vulvectomy) and lymph nodes in the groin. 
• Surgery is followed by radiation therapy and sometimes chemotherapy.
• Cancer can be treated with radiation (with or without chemo) initially, followed by surgery to remove any leftover cancer to protect normal tissues such as the vagina, urethra, and anus.
• Radiation and chemotherapy (without surgery) can be used as the primary treatment for women who are unable to undergo surgery due to other medical issues.

Stage IVA treatment

• The goal of surgery is to remove as much cancer as possible. The organ containing cancer cells determines whether the procedure should be prolonged. Another alternative is pelvic exenteration, which is rarely used.
• Conventional treatment involves a mix of surgery, radiation, and chemotherapy. Radiation treatment can be administered either before or after surgery. Chemotherapy can be administered before surgery.
• Radiation and potentially chemo can be administered to women who are unable to undergo surgery due to other medical issues.
• If the tumors have not migrated to neighboring organs but have spread to nearby lymph nodes, causing the lymph nodes to become adhered to the underlying tissue or ulcerated (become open sores), radical vulvectomy and excision of the groin lymph nodes can be performed. 

Stage IVB treatment

• There is no conventional therapy for cancer that has progressed to the pelvic lymph nodes or to organs and tissues outside the pelvis. 
• Although surgery is unlikely to cure the tumors, it may help reduce symptoms such as bowel or bladder obstructions. Radiation and chemotherapy can also help improve symptoms.
• Women can enroll themselves in ongoing chemical trials to get tested for new drugs that may help prolong their life.

The treatment of vulvar cancer itself may result in symptoms and long-term complications. Because the vulva has many blood and lymphatic vessels, cancer that begins here can quickly spread to other regions of the body, such as the vagina and bladder. 

Most vulvar cancer cases that are detected and treated early respond well to treatment. However, the prognosis is determined by the size of the tumor and whether the cancer has spread. Cancer often returns at or around the initial tumor location.

Practicing safe sex may reduce the risk of vulvar cancer. There is also a vaccine available to protect against specific types of HPV infection. The vaccine has been licensed for the prevention of cervical cancer and genital warts and may aid in the prevention of other malignancies associated with HPV, such as vulvar cancer. 

Routine pelvic examinations play an important role in the early detection of vulvar cancer. Early detection increases the likelihood of effective treatment. 

Nearly 87% of women with vulvar cancer that has not spread outside the vulva live for 5 years or more. If the disease spreads to other areas, about half of those diagnosed with vulvar cancer live for at least 5 years. When cancer spreads to distant places, such as the lungs and bones, that percentage reduces to roughly 22%. 

For these reasons, early detection and correct diagnosis are critical for assuring a favorable outcome.