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 is the 4th most common gynecologic cancer in the US; it accounts for 5% of cancers of the female genital tract; it caused an estimated 4700 new cases and 1000 deaths in 2013. Average age at diagnosis is about 70, and incidence increases with age. Incidence of vulvar cancer appears to be increasing in young women. 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 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 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 disease (a pale eczematoid lesion), Bartholin gland cyst, or condyloma acuminatum. Clinicians should consider vulvar cancer if a vulvar lesion develops in women at low risk of sexually transmitted diseases (STDs) or if it does not respond to treatment for STDs.

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.

Pearls & Pitfalls If a vulvar lesion develops in a woman at low risk of STDs or does not respond to treatment for STDs, consider vulvar cancer.

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 Confined to the vulva or perineum and no lymph node metastases > 90% IA ≤ 2 cm in all dimensions and < 1 mm of invasion IB > 2 cm in any dimension or > 1 mm of invasion II Tumor of any size with adjacent spread (lower third of the urethra, lower third of the vagina, or the anus) and no lymph node metastases 80% III Tumor of any size, with or without adjacent spread (lower third of the urethra, lower third of the vagina, or the anus), and with regional (inguinofemoral) lymph node metastases 50–60% IIIA 1 or 2 lymph node metastases, each < 5 mm or 1 lymph node metastasis of ≥ 5 mm IIIB 3 or more lymph node metastases, each < 5 mm or 2 or more lymph node metastases, each ≥ 5 mm IIIC Lymph node metastases with extracapsular spread IV Invasion of other regional structures (upper two thirds of the urethra, upper two thirds of the vagina, bladder mucosa, or rectal mucosa), is fixed to the pelvic bone, or has fixed or ulcerated regional (inguinofemoral) lymph nodes or distant metastases 15% IVA Invasion of the upper two thirds of the urethra, upper two thirds of the vagina, bladder mucosa, or rectal mucosa; is fixed to pelvic bone; or has fixed or ulcerated regional lymph nodes IVB Any distant metastases including in pelvic lymph nodes *Risk of lymph node spread is proportional to tumor size and invasion depth. Based on staging established by the International Federation of Gynecology and Obstetrics (FIGO) and American Joint Committee on Cancer (AJCC), AJCC Cancer Staging Manual, ed. 7. New York, Springer, 2010. Vulvar Cancer by Stage Stage Description 5-yr Survival Rate* I Confined to the vulva or perineum and no lymph node metastases > 90% IA ≤ 2 cm in all dimensions and < 1 mm of invasion IB > 2 cm in any dimension or > 1 mm of invasion II Tumor of any size with adjacent spread (lower third of the urethra, lower third of the vagina, or the anus) and no lymph node metastases 80% III Tumor of any size, with or without adjacent spread (lower third of the urethra, lower third of the vagina, or the anus), and with regional (inguinofemoral) lymph node metastases 50–60% IIIA 1 or 2 lymph node metastases, each < 5 mm or 1 lymph node metastasis of ≥ 5 mm IIIB 3 or more lymph node metastases, each < 5 mm or 2 or more lymph node metastases, each ≥ 5 mm IIIC Lymph node metastases with extracapsular spread IV Invasion of other regional structures (upper two thirds of the urethra, upper two thirds of the vagina, bladder mucosa, or rectal mucosa), is fixed to the pelvic bone, or has fixed or ulcerated regional (inguinofemoral) lymph nodes or distant metastases 15% IVA Invasion of the upper two thirds of the urethra, upper two thirds of the vagina, bladder mucosa, or rectal mucosa; is fixed to pelvic bone; or has fixed or ulcerated regional lymph nodes IVB Any distant metastases including in pelvic lymph nodes *Risk of lymph node spread is proportional to tumor size and invasion depth. Based on staging established by the International Federation of Gynecology and Obstetrics (FIGO) and American Joint Committee on Cancer (AJCC), AJCC Cancer Staging Manual, ed. 7. New York, Springer, 2010.

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 except when stromal invasion is < 1 mm
• Radiation therapy, chemotherapy, or both for stage III or IV cancer

Wide (≥ 2-cm margin) radical excision of the local tumor is indicated in all cases. Inguinal and femoral lymph node dissection can be done when stromal invasion is > 1 mm but is unnecessary when stromal invasion is < 1 mm. Recent studies suggest that sentinel lymph node biopsy is a reasonable alternative to lymph node dissection in some women with squamous cell carcinoma of the vulva. 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-fluorouracil, cisplatin), 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.

Key Points

• Most vulvar cancers are skin cancers (eg, squamous cell carcinoma, melanoma).
• Consider vulvar cancers if vulvar lesions, including pruritic lesions and ulcers, do not respond to treatment for STDs or occur in women at low risk of STDs.
• Diagnose vulvar cancer by biopsy, and stage it surgically.
• For cancers without distant metastases, use wide excision, and unless stromal invasion is < 1 mm, do lymph node dissection or sentinel lymph node biopsy.

Last full review/revision May 2013 by Pedro T. Ramirez, MD; David M. Gershenson, MD