Vulval Cancer

Vulval cancer is a cancer that develops on part of the female genitalia referred to as Vulval. Vulval includes the following structures from above downwards: the mons pubis, the clitoris, the urethral opening and the vaginal opening and the two skin folds on either sides; the labia major and labia minora. It is a rare cancer and accounts for only 4% of all the gynaecological cancers.

The causes of vulval cancer are not very clear, however, many avoidable or unavoidable factors have been identified that may increase the risk of the disease. These factors include:

• Age: Most of the cases of vulval cancer are seen in women over the age of 70 years. Less than 20% of cases are seen in women below 50 years of age.
• Exposure to Human papillomavirus (HPV): Women who have been infected with HPV are at a higher risk of having vulva cancer.
• Vulvar intraepithelial neoplasia (VIN): VIN is a precancerous condition, where the cells of the epithelia can develop into a squamous cell carcinoma over a period of years.
• Lichen sclerosis or atrophicus (LSA): Lichen sclerosis is an uncommon condition that creates patchy, white skin that appears thinner than normal. It usually affects the genital and anal areas.Anyone can get lichen sclerosis but postmenopausal women are at higher risk. These women are at an increased risk of squamous cell carcinoma of the affected area.
• Smoking: Smoking increases the risk of vulvar cancer.
• Weakened immune system: People who take medications to suppress the immune system, such as those who've undergone organ transplant, and those with conditions that weaken the immune system, such as human immunodeficiency virus (HIV) infection, have an increased risk of getting vulvar cancer.

• Squamous cell Carcinoma: About 90% of all cases of vulval cancer are of this type. This cancer is very often seen as a progression of Vulvar Intraepithelial Neoplasia or VIN, which is a precancerous condition. Squamous cell cancers can be caused by persistent infection with Human Papillomavirus or HPV and is more prevalent in younger women. Vulvar Dystrophy, which is more common in older women can also give rise to squamous cell carcinoma.
• Verrucous carcinoma is a subtype of the squamous cell cancer. It appears as a slowly growing wart.
• Vulvar Melanoma: This accounts for almost 5% of all cases of vulval cancer. Most vulvar melanomas are black or dark brown, but they can be white, pink or red. Vulvar melanomas can sometimes start in a mole, so a change in a mole that has been present for years can also indicate melanoma.
• Adenocarcinoma: This type of cancer originates in the glandular tissue, which lines the vulval and can present like a swelling or non-healing ulcer
• Sarcoma: Sarcoma originates in the connective tissues of the vulva. These are rare but aggressive cancer

Vulvar intraepithelial neoplasia (VIN): VIN is a precancerous condition. It most often presents with itching that does not go away or get better. An area of VIN may look different from normal vulvar skin. It is often thicker and lighter than the normal skin around it. However, an area of VIN can also appear red, pink, or darker than the surrounding skin. Because these changes are often caused by other conditions that are not pre-cancerous, some women don't realize that they might have a serious condition. Some try to treat the problem themselves with over-the-counter remedies. It is important that you consult your doctor without delay, so that with proper treatment, progression to cancer can be avoided.

• Burning sensation or persistent itching in the vulval region
• Pain while passing urine
• Straining while passing urine
• Bleeding or pain during or after intercourse
• Discoloration or swelling or ulcer in the vulval region
• Swelling or ulceration in the upper thigh region (inguinal regions)
• Wart-like growths

If you have any of these symptomsfor more than two weeks, you should consult a doctor.

Once, you see a specialist, the following tests may be carried out:

A Physical Examination: A through physical examination will be carried out for any abnormal growths or swellings, after taking a detailed history of your symptoms

Biopsy: A biopsy of the growth or swellingor anysuspicious area of the skin will be done after injecting local anaesthetic agent, so that the procedure is painless. The biopsy will be then be processed by the pathologists to give a histopathological report.

• If the area of suspicion is small, excisional biopsy may be performed i.e. the entire lesion or area will be removed and sent for histopathological examination.
• If the area of abnormal growth is large, a small punch biopsy is done and a small portion of the growth is taken for testing.

Imaging Tests:

• CECT scan: A CT scan of the abdomen and pelvis may be ordered to detect spread of cancer in pelvic or abdominal lymph nodes or to other organs like the liver.
• MRI: Sometimes a MRI scan may be ordered if the cancer is very close to the urethra or rectum
• PET CECT: A PET CT scan may be suggested in advanced or recurrent cases to rule out spread of cancer to other sites, eg lungs, liver, bone, etc.

HPV typing: It may be done in some cases, especially when cancers in cervix and/or vagina are also present along with vulval cancer. This test is done on a sample of cells from the patients’ cervix. Strains of human papillomavirus such as HPV 16 and HPV 18 are checked for.

The treatment for vulval cancer depends upon the stage and type of cancer. The treatment plan will most likely comprise of the following procedures:

Surgery:

• Excision of primary tumor: Excision entails removal of the primary tumor with a margin of normal tissue all around. Sometimes in case of large tumors a Vulvectomy may be performed. This procedure removes the vulval either in part or in entirety. There are of several types:
  
  • Skinning vulvectomy
  • Simple vulvectomy
  • Modified radical vulvectomy
  • Radical vulvectomy
• Pelvic exentration: This procedure is done to remove the vulva, surrounding lymph nodes, and one or more organs.
• Lymph node dissection: In some case, unilateral or bilateral lymph nodal dissection will be done, depending on the depth of the primary tumor and / or presence of spread to the lymph nodes.

Radiation therapy: It makes the use of high energy rays to target and destroy the cancer cells. Radiation treatment may be used as an alternative to surgery or after surgery as adjuvant treatment. Radiation treatment may be external or internal.

• In external radiation, the beams are aimed at the cancer site with the help of a machine.
• In internal radiation, the beams come from a capsule that contains some amount of radioactive material. These capsules are placed at the site of the primary tumor. And is also called implant radiation or brachytherapy.

Chemotherapy:Chemotherapy in the form of injections or tablets may be suggested depending on the stage of disease. It is done in 2 -3 weekly cycles, for a total of 6 cycles. It can also be recommended during radiation treatment; called as concurrent chemo-radiotherapy.

Biological therapy or immunotherapy:In advanced cases, biological therapy or immunotherapy may be suggested for treatment. Immunotherapy targets the cells, which affect the body’s immune response and helps the body in killing cancer cells.

• To reduce your risk of vulval cancer, reduce your risk of the sexually transmitted infection HPV. Use of a condom every time you have sex is protective.
• Get the HPV vaccine. Children and young adults may consider the HPV vaccine, which protects against the strains of the virus that are thought to cause the most cases of vulval cancer.
• Consult your doctor at regular intervals. These consultations allow your doctor to visually examine your vulval and manually examine your internal reproductive organs to check for abnormalities.