MALIGNANT TUMORS
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.
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| Plate 6-17 |
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.
