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