■ Vulvar Cancer

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Vulvar Cancer

Anne M. Noonan and Christina M. Annunziata

EPIDEMIOLOGY

■Vulvar cancer accounts for <5% of all female genital malignancies.

■A total of 4,490 new cases and 950 deaths from vulvar cancer were projected for 2012.

■It is most frequent in women in the seventh decade of life, but is occasionally diagnosed in women younger than 40 years.

■One in 368 women will be diagnosed with vulvar cancer during their lifetime (2.3 per 100,000 women per year in the United States).

ETIOLOGY AND RISK FACTORS

The etiology of vulvar cancer remains unclear, but potentially involves two distinct diseases associated with the following:

■Human papillomavirus (HPV) DNA, especially type 16

•Can be detected in 80% of intraepithelial lesions

•Found in 10% to 15% of invasive vulvar cancers (especially squamous cell)

■Chronic inflammation

•Venereal or granulomatous lesions

•Lichen sclerosus (coexists with up to 25% of vulvar cancers)

•Squamous hyperplasia (hyperplastic dystrophy) or lichen simplex chronicus

•Paget disease of the vulva (preinvasive)

Risk Factors

■Vulvar intraepithelial neoplasia (VIN), especially high grade (VIN III), increases the risk of development of invasive vulvar cancer.

■Other risk factors include prior history of cervical cancer, immunodeficiency disorders (e.g., HIV), and cigarette smoking.

■Classic risk factors such as hypertension, diabetes mellitus, and obesity are probably associated with aging and are not truly independent risk factors for this malignancy.

HISTOLOGY

■Squamous cell carcinomas (SCCs) constitute >90% of cases.

■Melanomas constitute <10% of cases.

■The remainder of tumor types include adenocarcinoma, basal cell carcinoma, verrucous carcinoma, sarcoma, clear cell carcinoma, and other rare tumors.

VULVAR SQUAMOUS CELL CARCINOMA

Vulvar SCC is commonly indolent, with slow extension and late metastases. Signs and symptoms in order of decreasing frequency are pruritus, mass, pain, bleeding, ulceration, dysuria, and discharge. Many patients are asymptomatic.

Diagnostic Workup

■Biopsy must include adequate tissue to determine histology and grade, depth of invasion, and stromal reaction present.

■Colposcopy using 5% acetic acid solution may be necessary to delineate suspected multifocal lesions.

■Cystoscopy, proctoscopy, chest x-ray, and intravenous urography should be performed as needed based on the extent of disease.

■Suspected bladder or rectal involvement must be biopsied.

■If invasive disease is present, detailed pelvic exam, CT, or MRI should be performed to assess deep and pelvic lymph nodes (LNs).

Indications for Excisional Biopsy of Vulvar Lesions

■Any gross lesion

■Red, white, dark brown, or black skin patches

■Areas firm to palpation

■Pruritic, tingling, or bleeding lesions

■Any nevi in the genital tract

■Enlarged or thickened areas of Bartholin glands, especially in postmenopausal women

Location and Metastatic Spread Pattern of Vulvar SCC

■Vulvar SCC is found on

•The labia majora in 50% of cases

•The labia minora in 15% to 20% of cases

•The clitoris and perineum in rare cases

■Vulvar SCC tends to grow locally, with subsequent spread to inguinal, femoral, and pelvic LNs.

■Hematogenous spread rarely occurs without LN involvement.

Staging

■Vulvar cancer is a surgically staged disease. The revised 2009 FIGO staging system is as follows:

•Stage I: Tumor confined to the vulva

•IA: Lesions ≤2 cm in size, confined to the vulva or perineum, and with stromal invasion ≤1.0 mm, no nodal metastasis

•IB: Lesions >2 cm in size or with stromal invasion >1.0 mm, confined to the vulva or perineum, and with negative nodes

•Stage II: Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with negative nodes

•Stage III: Tumor of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguinofemoral LNs

•IIIA: (i) With one LN metastasis (≥5 mm), or (ii) 1 to 2 LN metastasis(es) (<5 mm)

•IIIB: (i) With two or more LN metastases (≥5 mm), or (ii) three or more LN metastases (<5 mm)

•IIIC: With positive nodes with extracapsular spread

•Stage IV: Tumor invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures

•IVA: Tumor invades any of the following: (i) upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or (ii) fixed or ulcerated inguinofemoral LNs

•IVB: Any distant metastasis including pelvic LNs

Prognosis and Survival

■Survival depends on stage, LN involvement, depth of invasion, structures involved, and tumor location.

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