Vulvar cancer classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Musadiq Ali M.B.B.S.[2] Monalisa Dmello, M.B,B.S., M.D. [3]

Overview

Vulvar cancer may be classified according to histology:Squamous cell carcinoma, Basal cell carcinoma, Vulvar malignant melanoma Vulvar sarcoma, Vulvar Paget disease, Bartholin gland carcinoma.

Classification

Histologic subtypes of vulvar cancer include:

• Squamous cell carcinoma
  • Verrucous carcinoma
• Basal cell carcinoma
• Vulvar malignant melanoma
• Vulvar sarcoma
• Vulvar Paget disease
• Bartholin gland carcinoma

Squamous cell carcinoma

• Seventy-five percent or more of vulvar malignancies are squamous cell carcinomas [2,3]. There are two subtypes, both of which usually occur on the labia or vestibule:
• The keratinizing, differentiated, or simplex type is more common. This occurs in older women and is not related to human papillomavirus (HPV) infection, but is associated with vulvar dystrophies such as lichen sclerosus and, in developing countries, chronic venereal granulomatous disease.
• The classic, warty, or Bowenoid type is predominantly associated with HPV 16, 18, and 33, and found in younger women [17,18].These women tend to present with early-stage disease [19], although several cases of stage III/IV disease in HIV-infected women have been reported [20].
• There is evidence that some high-grade vulvar and vaginal intraepithelial neoplasias are monoclonal lesions derived from high-grade or malignant cervical disease [22].
• Presence of the cervix does not appear to be necessary for oncogenic HPV to infect the genital tract.

Verrucous carcinoma

• Verrucous vulvar carcinoma is a variant of squamous cell carcinoma that has distinctive features.
• Although cauliflower-like in appearance, it is differentiated from squamous cell carcinoma with a verrucous configuration.
• Biopsy shows papillary fronds without the central connective tissue core typical of condylomata acuminata.
• The lesion grows slowly and rarely metastasizes to lymph nodes, but it may be locally destructive.

Basal cell carcinoma

• Basal cell carcinoma is a squamous histology, but is distinct from squamous cell vulvar carcinoma.
• Approximately 2 to 8 percent of vulvar cancers are basal cell cancers, and 2 percent of basal cell cancers occur on the vulva [2,24].
• Basal cell vulvar carcinoma usually affects postmenopausal white women and may be locally invasive, although it is usually non-metastasizing [25,26].
• The typical appearance is that of a "rodent" ulcer with rolled edges and central ulceration.
• It is often asymptomatic, but pruritus, bleeding, or pain may occur.
• Basal cell carcinomas are associated with a high incidence of antecedent or concomitant malignancy elsewhere in the body [26].

Melanoma

• Melanoma is the second most common vulvar cancer histology, accounting for approximately 2 to 10 percent of primary vulvar neoplasms [25,27-29].
• Melanoma of the vulva occurs predominantly in postmenopausal, white, non-Hispanic women, at a median age of 68 years[28]. By contrast, cutaneous melanomas presenting at other sites often develop before age 45.
• Vulvar melanoma is usually a pigmented lesion, but amelanotic lesions also occur.
• Most arise de novo on the clitoris or labia minora, but can also develop within preexisting junctional or compound nevi [31].

Sarcoma

• Soft tissue sarcomas (including leiomyosarcomas, rhabdomyosarcomas, liposarcomas, angiosarcomas, neurofibrosarcomas, epithelioid sarcomas, and undifferentiated/unclassified soft tissue sarcomas) constitute 1 to 2 percent of vulvar malignancies. The prognosis is generally poor [32,33].
• As with soft tissue sarcomas located elsewhere on the extremities and trunk, high-grade lesions that are larger than 5 cm in diameter, with infiltrating margins and a high mitotic rate, are those most likely to recur.

Paget disease of the vulva

• Extramammary Paget disease, an intraepithelial adenocarcinoma, accounts for less than 1 percent of all vulvar malignancies [34]. Most patients are in their 60s and 70s and white.
• Pruritus is the most common symptom, present in 70 percent of patients. Vulvar Paget disease is similar in appearance to Paget disease of the breast. The lesion has an eczematoid appearance; it is well-demarcated and has slightly raised edges and a red background, often dotted with small, pale islands. It is usually multifocal and may occur anywhere on the vulva, mons, perineum/perianal area, or inner thigh. (See "Paget disease of the breast".)
• Diagnosis is based upon characteristic histopathology (picture 3A-B). Vulvar biopsy should be performed in patients with suspicious lesions, including those with persistent pruritic eczematous lesions that fail to resolve within six weeks of appropriate antieczema therapy.
• Invasive adenocarcinomas may be present within or beneath the surface lesion (4 to 17 percent in two series totaling 176 patients) (picture 4A-B) [34,35].
• Women with Paget disease of the vulva should also be evaluated for the possibility of synchronous neoplasms, as approximately 20 to 30 percent of these patients have a noncontiguous carcinoma (eg, involving breast, rectum, bladder, urethra, cervix, or ovary) [36].

Bartholin gland carcinoma

• Bartholin gland carcinoma comprises approximately 0.1 to 5 percent of all vulvar carcinomas and 0.001 percent of all female malignancies [37]. *The incidence of Bartholin gland carcinoma in one series was 0.023 per 100,000 woman-years in premenopausal women and 0.114 per 100,000 woman-years in postmenopausal women [38]. The incidence of Bartholin gland carcinoma is highest among women in their 60s. Most affected women do not have a past history of benign Bartholin gland disorders.
• Cancers arising in the Bartholin gland are most often adenocarcinomas or squamous cell carcinomas, but transitional cell carcinomas, adenosquamous, and adenoid cystic carcinomas may also develop [39,40]. Most primary adenocarcinomas of the vulva occur in the Bartholin gland. *Only the squamous cell carcinomas of the Bartholin gland are related to HPV infection [39].
• Metastatic disease is common in cancers of the Bartholin gland because of the rich vascular and lymphatic network in this area. In one series of 11 women with Bartholin gland cancer, 55 percent developed recurrent disease, and 67 percent were alive at five years [41]. The only two patients with isolated vulvar recurrence were alive without disease at 8 and 180 months, respectively.