About 5% of the malignant tumors of the female genital organs originate on the vulva. (The incidence of vulvar cancer rose by approximately 20% between 1973 and 2000, likely related to increased exposure to human papillomavirus [HPV].) Primary carcinoma is almost always seen in elderly women with an average age for in situ tumors being 40 to 49 years, and 65 to 70 for invasive lesions. The vast majority of these tumors are of the squamous cell variety. Histologic types include squamous cell (90%), melanoma (5%), basaloid, warty, verrucous, giant cell, spindle cell, acantholytic squamous cell (adenoid squamous), lymphoepithelioma-like, basal cell, and Merkel cell. Sarcoma accounts for approximately 2% of vulvar cancers. Metastatic tumors from other sources are rare but do occur.
Squamous cell cancer of the vulva generally presents as an exophytic ulcer or hyperkeratotic plaque. It may arise as a solitary lesion or develop hidden within hypertrophic or other vulvar skin changes, making diagnosis difﬁcult and often delayed. Known or suspected risk factors include infection with human papillomavirus (molecular analysis had detected HPV DNA in 40% of vulvar cancers), smoking, immunosuppression, and lichen sclerosus.
Occasionally, adenocarcinoma may develop from Bartholin gland, mucous glands, or sweat glands. Rarely, a medullary carcinoma may be seen. The sites of origin, in the order of their frequency, are the labia majora, prepuce of the clitoris, labia minora, Bartholin gland, posterior commissure, and urethral area.
Leukoplakia and venereal granulomatous lesions appear to be predisposing factors in the development of vulvar malignancy. It is estimated that about 50% of primary carcinomas are preceded by leukoplakia. The initial lesion may be a small, ﬁrm nodule or thickening, with slow but progressive enlargement, inﬁltration and, ﬁnally, ulceration. The early symptoms may be insigniﬁcant, consisting merely of soreness and pruritus. In the neglected case the tumor may become large, nodular, hypertrophic, ulcerated, and foul smelling. Additional prevailing complaints may then include a purulent, odoriferous leukorrhea and local irritation following urination. Lymphatic extension to the regional inguinal nodes occurs early and in a high percentage of cases. Distant metastases are rare. However, because pulmonary involvement is occasionally encountered, a routine x-ray examination of the chest is warranted. Because of neglect and lack of recognition, the average case is not brought to operation until about 1 year after the onset of symptoms.
Basal cell carcinoma of the vulva is relatively uncommon. A variable incidence of 1.2% to 13% of the epidermoid carcinomas has been reported. Basal cell carcinoma of the vulva is to be differentiated from the squamous cell variety. The age of appearance, the signs, and the symptoms are similar to those of early squamous cell carcinomas. A rodent ulcer or superﬁcial erythematous type may be seen. Deﬁnite connections with other diseases or predisposing factors, such as leukoplakia or hypertrophic venereal lesions, have not been established. The neoplasms are slow-growing and radiosensitive. Regional metastases are rare, but local extension and recurrence are characteristic. Wide local excision may sufﬁce instead of the more radical vulvectomy and bilateral femoral and pelvic lymphadenectomy.
Secondary carcinoma of the vulva (metastatic to) is uncommon but may occur. This is particularly true of metastases from a hypernephroma of the kidney, chorioepithelioma of the uterus, and carcinoma of the uterine body or cervix. At times, the vulvar lesion may be the ﬁrst indication of the existence of a primary carcinoma elsewhere.
Sarcoma of the vulva is infrequent. Varieties include ﬁbrosarcoma, spindle-cell sarcoma, lymphosarcoma, myxosarcoma, liposarcoma, round cell, giant cell, and polymorphous cell sarcoma. These are usually very aggressive in character. Occasionally, their malignancy may be of low grade.