Vulvar Cancer

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:

Vulvar cancer may mimic sexually transmitted genital ulcers (eg, 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... 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 Cyst and Bartholin Gland Abscess Bartholin gland cysts are the most common large vulvar cysts. They are mucus-filled and are located on either side of the vaginal opening. Symptoms of large cysts include vulvar pressure or... read more or abscess, or condyloma acuminatum (genital wart) Human Papillomavirus (HPV) Infection Human papillomavirus (HPV) infects epithelial cells. Most of the > 100 subtypes infect cutaneous epithelium and cause skin warts; some types infect mucosal epithelium and cause anogenital warts... read more . Clinicians should consider vulvar cancer if a vulvar lesion develops in women at low risk of sexually transmitted infections (STIs) or if it does not respond to treatment for STIs.

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.

(See also National Comprehensive Cancer Network (NCCN): NCCN Clinical Practice Guidelines in Oncology: Vulvar Cancer [Squamous Cell Carcinoma].)

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 lateralized lesions ≤ 2 cm, unilateral wide local excision and unilateral SLN dissection is recommended. Lesions near the midline and most lesions > 2 cm require bilateral SLN dissection.

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. SLN mapping should not be considered if clinical findings suggest cancer has spread to lymph nodes in the groin. For SLN 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.

Further evaluation of lymph nodes and treatment are based on the results of SLN biopsy or lymphadenectomy. If inguinofemoral nodes are negative, observation is reasonable. If one or more SLNs are positive, treatment depends on the size of the metastasis. For micrometastases (≤ 2 mm), inguinofemoral radiotherapy (50 Gy) is a safe alternative to inguinal lymphadenectomy. For macrometastases in an SLN (> 2 mm), inguinal lymphadenectomy is recommended to prevent isolated groin recurrence, although inguinal lymphadenectomy is associated with significant risks of morbidity, wound complications, lymphedema, and lymphoceles (1 Treatment references Vulvar cancer is usually a squamous cell cancer, most often occurring in older women. It usually manifests as a visible or palpable lesion. Diagnosis is by biopsy. Treatment typically includes... read more ). An ongoing, single-arm, prospective phase II trial is evaluating the safety of replacing inguinofemoral lymphadenectomy with chemoradiation (56 Gy) in patients with early-stage vulvar cancer with a macrometastasis (> 2 mm) and/or extracapsular extension in the sentinel node (2 Treatment references Vulvar cancer is usually a squamous cell cancer, most often occurring in older women. It usually manifests as a visible or palpable lesion. Diagnosis is by biopsy. Treatment typically includes... read more ).

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 (cisplatin, carboplatin, cisplatin or carboplatin/paclitaxel, or cisplatin/paclitaxel plus bevacizumab).

Overall 5-year survival rate of vulvar cancer is approximately 70%. 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.

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