Vulvar cancer is usually a squamous cell skin cancer, most often occurring in elderly women. It usually manifests as a palpable lesion. Diagnosis is by biopsy. Treatment includes excision and inguinal and femoral lymph node dissection.

Vulvar cancer accounts for about 3 to 4% of gynecologic cancers in the US. Average age at diagnosis is about 70, and incidence increases with age. Risk factors include vulvar intraepithelial neoplasia (VIN), human papillomavirus infection, heavy cigarette smoking, lichen sclerosus, squamous hyperplasia, squamous carcinoma of the vagina or cervix, and chronic granulomatous diseases.

Pathology

VIN is a precursor to vulvar cancer. VIN may be multifocal. Sometimes adenocarcinoma of the vulva, breast, or Bartholin's glands also develops.

About 90% of vulvar cancers are squamous cell carcinomas; about 5% are melanomas. Others include adenocarcinomas and transitional cell, adenoid cystic, and adenosquamous carcinomas; all may originate in Bartholin's glands. Sarcomas and basal cell carcinomas with underlying adenocarcinoma also occur.

Vulvar cancer may spread by direct extension (eg, into the urethra, bladder, vagina, perineum, anus, or rectum), hematogenously, to the inguinal lymph nodes, or from the inguinal lymph nodes to the pelvic and para-aortic lymph nodes.

Symptoms and Signs

The most common presentation is a palpable vulvar lesion, frequently noticed by the woman or by a clinician during pelvic examination. Women often have a long history of pruritus. They may not present until cancer is advanced. The lesion may become necrotic or ulcerated, sometimes resulting in bleeding or a watery vaginal discharge. Melanomas may appear bluish black, pigmented, or papillary.

Diagnosis

• Biopsy
• Surgical staging

Vulvar cancer may mimic sexually transmitted genital ulcers (see Table: Sexually Transmitted Diseases (STDs): Chancroid), basal cell carcinoma, vulvar Paget's disease (a pale eczematoid lesion), Bartholin's gland cyst, or condyloma acuminatum. A dermal punch biopsy using a local anesthetic is usually diagnostic. Occasionally, wide local excision is necessary to differentiate VIN from cancer. Subtle lesions may be delineated by staining the vulva with toluidine blue or by using colposcopy.

Staging: Staging is based on tumor size and location and on regional lymph node spread as determined by lymph node dissection done as part of initial surgical treatment (see Table 9: Gynecologic Tumors: Vulvar Cancer by Stage).

Table 9
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Vulvar Cancer by Stage Stage Description 5-yr Survival Rate* I ≤ 2 cm in all dimensions and confined to the vulva or perineum without nodal metastases Ia: < 1 mm of invasion Ib: > 1 mm of invasion > 90% II Same as stage I except tumors are > 2 cm in any dimension 80% III Spread to the adjacent lower urethra, vagina, or anus or metastasized to unilateral regional lymph node 50–60% IV Invasion of the upper urethra, bladder mucosa, intestinal or rectal mucosa, pelvic bone, bilateral lymph nodes, or other distant tissues 15% * Risk of lymph node spread is proportional to tumor size and invasion depth.

Prognosis

Overall 5-yr survival rates depend on stage. Risk of lymph node spread is proportional to the tumor size and invasion depth. Melanomas metastasize frequently, depending mostly on invasion depth but also on tumor size.

Treatment

• Wide excision and lymph node dissection for all cancers
• Radiation therapy, chemotherapy, or both for stage III or IV cancer

Wide (≥ 2-cm margin) radical excision of the local tumor and a unilateral or bilateral inguinal and femoral lymph node dissection are necessary for cancers of all stages and are sufficient for stages I and II. For lateralized lesions ≤ 2 cm, unilateral wide local excision and unilateral lymph node dissection can be used. Lesions near the midline and most lesions > 2 cm require bilateral lymph node dissection.

For stage III, lymph node dissection followed by postoperative external beam radiation therapy, often with chemotherapy (eg, 5-fluorouracilSome Trade Names
ADRUCIL
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, cisplatinSome Trade Names
PLATINOL
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), is usually done before wide radical excision. The alternative is more radical or exenterative surgery.

For stage IV, treatment is some combination of pelvic exenteration, radiation therapy, and systemic chemotherapy.

Last full review/revision November 2008 by David M. Gershenson, MD; Pedro T. Ramirez, MD

Content last modified February 2012