Vulvar cancer

Overview

Vulvar cancer is a malignant, invasive growth in the vulva, or the outer portion of the female genitals. The disease accounts for only 0.6% of cancer diagnoses but 5% of gynecologic cancers in the United States. The labia majora are the most common site involved representing about 50% of all cases, followed by the labia minora. The clitoris and Bartholin glands may rarely be involved. Vulvar cancer is separate from vulvar intraepithelial neoplasia (VIN), a superficial lesion of the epithelium that has not invaded the basement membrane—or a pre-cancer. VIN may progress to carcinoma-in-situ and, eventually, squamous cell cancer.

According to the American Cancer Society, in 2014, there were about 4,850 new cases of vulvar cancer and 1,030 deaths from the disease. In the United States, five-year survival rates for vulvar cancer are around 70%.

Symptoms

Typically, a lesion presents in the form of a lump or ulcer on the labia majora and may be associated with itching, irritation, local bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. The labia minora, clitoris, perineum and mons are less commonly involved. Due to modesty or embarrassment, patients may put off seeing a doctor.

Melanomas tend to display the typical asymmetry, uneven borders and dark discoloration as do melanomas in other parts of the body.

Adenocarcinoma can arise from the Bartholin gland and present with a painful lump.

Causes

The cause of vulvar cancer is unclear; however, some conditions such as lichen sclerosus, squamous dysplasia or chronic vulvar itching may precede cancer. In younger women affected with vulvar cancer, risk factors include low socioeconomic status, human papillomavirus (HPV) infection, multiple sexual partners, cigarette use and cervical cancer. Patients that are infected with HIV tend to be more susceptible to vulvar cancer as well.

Prevention

Reduce the risk of sexually transmitted infenction

To reduce the risk of vulvar cancer, reduce the risk of sexually transmitted infections such as HPV and HIV. To reduce the risk of these diseases:

• Limited number of sexual partners. more sexual partners are,greater the risk of exposure to HPV.
• Using a condom every time having sex. A condom can protect from HIV transmission. Condoms may reduce the risk of contracting HPV but can't fully protect against it.
• Get the HPV vaccine. Girls and young women should consider the HPV vaccine, which protects against the strains of the virus that are thought to cause the most cases of vulvar cancer.

Pelvic exams

Ask your doctor how often you should undergo pelvic exams. These exams allow your doctor to visually examine your vulva and manually examine your internal reproductive organs to check for abnormalities.

Talk to your doctor about your risk factors for vulvar cancer and other pelvic cancers in order to determine the most appropriate screening exam schedule for you.

Diagnosis

Examination of the vulva is part of the gynecologic evaluation and should include a thorough inspection of the perineum, including areas around the clitoris and urethra, and palpation of the Bartholin's glands. The exam may reveal an ulceration, lump or mass in the vulvar region. Any suspicious lesions need to be sampled, or biopsied. This can generally be done in an office setting under local anesthesia. Small lesions can be removed under local anesthesia as well. Additional evaluation may include a chest X-ray, an intravenous pyelogram, cystoscopy or proctoscopy, as well as blood counts and metabolic assessment.

Staging

Anatomical staging supplemented preclinical staging starting in 1988. FIGO’s revised TNM classification system uses tumor size (T), lymph node involvement (N) and presence or absence of metastasis (M) as criteria for staging. Stages I and II describe the early stages of vulvar cancer that still appear to be confined to the site of origin. Stage III cancers include greater disease extension to neighboring tissues and inguinal lymph nodes on one side. Stage IV indicates metastatic disease to inguinal nodes on both sides or distant metastases.

Prognosis

Overall, five-year survival rates for vulvar cancer are around 78% but may be affected by individual factors including cancer stage, cancer type, patient age and general medical health. Five-year survival is greater than 90% for patients with stage I lesions but decreases to 20% when pelvic lymph nodes are involved. Lymph node involvement is the most important predictor of prognosis. Thus, early diagnosis is important.

Treatment

Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. Surgery is a mainstay of therapy depending on anatomical staging and is usually reserved for cancers that have not spread beyond the vulva. Surgery may involve a wide local excision, radical partial vulvectomy, or radical complete vulvectomy with removal of vulvar tissue, inguinal and femoral lymph nodes. In cases of early vulvar cancer, the surgery may be less extensive and consist of wide excision or a simple vulvectomy. Surgery is significantly more extensive when the cancer has spread to nearby organs such as the urethra, vagina, or rectum. Complications of surgery include wound infection, sexual dysfunction, edema and thrombosis, as well as lymphedema secondary to dissected lymph nodes.

Sentinel lymph node (SLN) dissection is the identification of the main lymph node(s) draining the tumor, with the aim of removing as few nodes as possible, decreasing the risk of adverse effects. Location of the sentinel node(s) may require the use of technetium(99m)-labeled nano-colloid, or a combination of technetium and 1% isosulfan blue dye, wherein the combination may reduce the number of women with "'missed"' groin node metastases compared with technetium only.

Radiation therapy may be used in more advanced vulvar cancer cases when disease has spread to the lymph nodes and/or pelvis. It may be performed before or after surgery. Chemotherapy is not usually used as primary treatment but may be used in advanced cases with spread to the bones, liver or lungs. It may also be given at a lower dose together with radiation therapy.

Women with vulvar cancer should have routine follow-up and exams with their oncologist, often every 3 months for the first 2–3 years after treatment. They should not have routine surveillance imaging to monitor the cancer unless new symptoms appear or tumor markers begin rising. Imaging without these indications is discouraged because it is unlikely to detect a recurrence or improve survival and is associated with its own side effects and financial costs.