Vulvar Cancer

Essentials of Diagnosis

• Two independent pathways for development: HPV or chronic inflammation

• History of prolonged vulvar irritation, with pruritus, local discomfort, or slight bloody discharge

• Early lesions may suggest or include nonneoplastic epithelial disorders

• Late lesions appear as a mass, an exophytic growth, or a firm, ulcerated area in the vulva

• Biopsy is necessary for diagnosis

General Considerations

• The majority of cancers of the vulva are squamous lesions; basal cell carcinomas are also found

• Several subtypes (particularly 16, 18, and 31) of the human papillomavirus (HPV) have been identified in some but not all vulvar cancers

• About 70–90% of vulvar intraepithelial neoplasia (VIN) and 40–60% of vulvar cancers are HPV associated

• Vulvar lichen sclerosus also is associated with an increased risk of developing vulvar cancer

• As with squamous cell lesions of the cervix, a grading system of VIN from mild dysplasia to carcinoma in situ is used

Demographics

• Usually occurs in women over 50 years of age

• The likelihood that a superimposed vulvar cancer will develop in a woman with a nonneoplastic epithelial disorder (vulvar dystrophy) is 1–5%

Symptoms and Signs

• Early lesions may suggest or include nonneoplastic epithelial disorders

• Late lesions appear as

  • A mass

  • An exophytic growth

  • A firm, ulcerated area in the vulva

Differential Diagnosis

• Vulvar intraepithelial neoplasia

• Chronic granulomatous lesions (eg, lymphogranuloma venereum, syphilis), condylomas, hidradenoma, or neurofibroma

• Genital warts (condyloma acuminata)

• Lichen sclerosus

• Psoriasis

• Lichen planus

• Epidermal inclusion cysts

• Hidradenomas, or neurofibromas

Laboratory Tests

• Pathologic examination of biopsies of lesion(s)

Imaging Studies

• Preoperative colposcopy of vulva, vagina, and cervix

• CT or MRI of the pelvis or abdomen is generally not required except in advanced cases for planning therapeutic options

Diagnostic Procedures

• Biopsy is essential for the diagnosis and should be performed with any localized atypical vulvar lesion, including white patches

• Multiple skin-punch specimens can be taken in the office under local anesthesia, with care to include tissue from the edges of each lesion sampled

• Lichen sclerosus and other associated leukoplakic changes in the skin should be biopsied

Medications

• A 7:3 combination of betamethasone and crotamiton is particularly effective for itching

• After an initial response, fluorinated steroids should be replaced with hydrocortisone because of their skin atrophying effect

• For lichen sclerosus

  • Apply clobetasol propionate cream 0.05% twice daily for 2–3 weeks, then once daily until symptoms resolve

  • Application one to three times a week can be used for long-term maintenance therapy

Surgery

• In situ carcinoma

  • In situ squamous cell carcinoma of the vulva and small, invasive basal cell carcinoma of the vulva should be excised with a wide margin

  • If the squamous carcinoma in situ is extensive or multicentric, laser therapy or superficial surgical removal of vulvar skin may be required

    • In this way, the clitoris and uninvolved portions of the vulva may be spared

    • Skin grafting may be necessary, but mutilating vulvectomy is avoided

• Invasive carcinoma

  • Invasive carcinoma confined to the vulva without evidence of spread to adjacent organs or to the regional lymph nodes is treated with wide local excision (WLE) and inguinal lymphadenectomy or WLE alone if invasion is < 1 mm

  • Patients with more advanced disease may receive preoperative radiation, chemotherapy, or both

• Sentinel lymph node sampling is the standard of care for women with early stage vulvar cancer

Follow-Up

• Examination every 3 months for 2 years postoperatively, then every 6 months for an additional 3 years

Complications

• Depends on extent of surgery

• Wound infection

• Wound breakdown

• Lymphedema may occur as a late complication in up to 30%

Prognosis

• Basal cell vulvar carcinomas very seldom metastasize; with adequate excision, the prognosis is excellent

  • Patients with invasive vulvar carcinoma ≤ 2 cm in diameter without inguinal lymph node metastases have an 85–90% 5-year survival rate

  • If the lesion is > 2 cm and lymph node involvement is present, the likelihood of 5-year survival is ~40%

Prevention

• Careful examination of the vulva for premalignant lesions at all gynecologic examinations

When to Refer

• All patients with invasive vulvar carcinoma should be referred to a gynecologic oncologist