Vulvar cancer is the 4th most common gynecologic cancer in the US; it accounts for 5% of cancers of the female genital tract. In the US, vulvar cancer will cause an estimated 6120 new cases and 1350 deaths in 2020.
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 for vulvar cancer include
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 as follows:
Most patients with vulvar cancer present with 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 (chancroid Chancroid Chancroid is infection of the genital skin or mucous membranes caused by Haemophilus ducreyi and characterized by papules, painful ulcers, and enlargement of the inguinal lymph nodes leading... read more ), basal cell carcinoma Basal Cell Carcinoma Basal cell carcinoma is a superficial, slowly growing papule or nodule that derives from certain epidermal cells. Basal cell carcinomas arise from keratinocytes near the basal layer, which are... read more , vulvar Paget disease (a pale eczematoid lesion), Bartholin gland cyst Bartholin Gland Cysts Bartholin gland cysts are mucus-filled and occur on either side of the vaginal opening. They are the most common large vulvar cysts. Symptoms of large cysts include vulvar irritation, dyspareunia... read more , or condyloma acuminatum Human Papillomavirus (HPV) Infection Human papillomavirus (HPV) causes warts. Some types cause skin warts, and other types cause raised or flat genital warts (lesions of the skin or mucous membranes of the genitals). Infection... read more . 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 of vulvar cancer 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 Vulvar Cancer by Stage Vulvar Cancer by Stage Vulvar cancer is usually a squamous cell skin cancer, most often occurring in older women. It usually manifests as a palpable lesion. Diagnosis is by biopsy. Treatment typically includes excision... read more ).
(See also National Comprehensive Cancer Network (NCCN): NCCN Clinical Practice Guidelines in Oncology: Vulvar Cancer.)
Wide (≥ 2-cm margin) radical excision of the local tumor is indicated when the tumor confined to the vulva with no extension to adjacent perineal structures. Lymph node dissection may be done when stromal invasion is > 1 mm but is unnecessary when stromal invasion is < 1 mm. Radical vulvectomy is usually reserved for Bartholin gland adenocarcinoma.
For tumors with extension to adjacent perineal structures such as the urethra, vagina, or anus, a modified radical vulvectomy is indicated independent of tumor size.
Sentinel lymph node biopsy is a reasonable alternative to lymph node dissection for some women with squamous cell vulvar carcinoma. Sentinel lymph node mapping should not be considered if clinical findings suggest cancer has spread to lymph nodes in the groin. For sentinel lymph node mapping, a tracer (blue dye, technetium-99 [99Tc], indocyanine green [ICG]) is injected intradermally around and in front of the leading edge of the vulvar carcinoma.
For lateralized lesions ≤ 2 cm, unilateral wide local excision and unilateral sentinel lymph node dissection is recommended. Lesions near the midline and most lesions > 2 cm require bilateral sentinel lymph node dissection.
For stage III, lymph node dissection followed by postoperative external beam radiation therapy and chemotherapy (chemoradiation)—preferably cisplatin; possibly fluorouracil—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.
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 the lesions occur in women at low risk of STDs.
Diagnose vulvar cancer by biopsy, and stage it surgically.
For cancers without distant metastases, use wide local excision, and unless stromal invasion is < 1 mm, do lymph node dissection or sentinel lymph node mapping.
For cancers with lymph node or distant metastases, treat with a combination of surgery, radiation therapy, and/or chemotherapy.
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
National Cancer Institute: Vulvar Cancer: This web site provides links to information about causes, prevention, and treatment of vulvar cancer, as well as links to information about screening, statistics, and supportive and palliative care.