Vulvar cancer: Clinical manifestations, diagnosis, and

pathology

Vulvar cancer: Clinical manifestations, diagnosis, and pathology

Authors
John C Elkas, MD, JD
Jonathan S Berek, MD, MMS
Section Editors
Barbara Goff, MD
Arno J Mundt, MD
Deputy Editor
Sandy J Falk, MD
Disclosures
All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Oct 2012. | This topic last updated: Apr 16, 2012.
INTRODUCTION Vulvar cancer is the fourth most common gynecologic cancer
(following cancer of the uterine corpus, ovary, and cervix) and comprises 5 percent of
malignancies of the female genital tract [1]. Annually in the United States, there are
approximately 4340 new cases and 940 deaths from this disease. Although the rate of
invasive vulvar carcinoma has remained stable over the past two decades, the incidence
of in situ disease (vulvar intraepithelial neoplasia) has more than doubled.
The clinical presentation, diagnosis, histology, and staging evaluation of women with
vulvar cancer will be reviewed here. Treatment and prognosis are discussed separately.
(See "Vulvar cancer: Staging, treatment, and prognosis".)
EPIDEMIOLOGY AND RISK FACTORS The age-adjusted incidence of vulvar cancer
in the United States was 2.5 per 100,000 women, based upon 1997 to 2004 data from a
national cancer database [2]. Vulvar carcinoma is encountered most frequently in
postmenopausal women. The mean age at diagnosis is 65, but may be falling. This was
illustrated in a study of 78 women diagnosed with vulvar cancer between 1979 and 1993
in which the average age at presentation dropped from 69 to 55 during this interval [3].
Risk factors for vulvar cancer include cigarette smoking, vulvar dystrophy (eg, lichen
sclerosus), vulvar or cervical intraepithelial neoplasia, human papillomavirus (HPV)
infection, immunodeficiency syndromes, a prior history of cervical cancer, and northern
European ancestry [4,5].
Two independent pathways of vulvar carcinogenesis are felt to currently exist, the first
related to mucosal HPV infection and the second related to chronic inflammatory (vulvar
dystrophy) or autoimmune processes [6]. HPV has been shown to be responsible for 60
percent of vulvar cancers [7]. Specifically, HPV 16 and 33 are the predominant subtypes
accounting for 55.5 percent of all HPV-related vulvar cancers [8].
The increasing incidence of HPV-related vulvar intraepithelial neoplasia among young
women may account for the fall in mean age of diagnosis of vulvar cancer discussed
above. HPV DNA is found more commonly in vulvar cancers of young women who smoke
as compared to older nonsmokers [7]. Early detection and treatment of vulvar
intraepithelial neoplasia may prevent the development of cancer. This may explain why

the incidence of invasive vulvar cancer has remained stable even though the incidence of
vulvar intraepithelial neoplasia has increased. (See "Vulvar intraepithelial neoplasia".)
CLINICAL MANIFESTATIONS The signs and symptoms of all histological types of
vulvar malignancy are similar. Most patients present with a unifocal vulvar plaque, ulcer,
or mass (fleshy, nodular, or warty) on the labia majora; the labia minora, perineum,
clitoris, and mons are less frequently involved (picture 1 and picture 2 and picture 3). In
10 percent of cases, the lesion is too extensive to determine the actual site of origin
(picture 4) [9].
Lesions are multifocal in 5 percent of cases; thus, all vulvar and perianal skin surfaces,
as well as the cervix and vagina, should be evaluated. A synchronous second
malignancy, most commonly cervical neoplasia, is found in up to 22 percent of patients
with a vulvar malignancy [10].
Pruritus is a common complaint associated with most vulvar disorders; it is especially
prevalent when there is an underlying vulvar dystrophy (eg, lichen sclerosus or
squamous cell hyperplasia). We biopsy patients with lichen sclerosus if there is a
suspicious lesion or the lesions are refractory to topical therapy.
Vulvar bleeding or discharge, dysuria, or an enlarged lymph node in the groin are less
frequently encountered symptoms, and suggestive of advanced disease. On the other
hand, many patients are asymptomatic at the time of diagnosis. (See "Dermatitis of the
vulva" and "Vulvar lichen sclerosus".)
DIAGNOSIS Vulvar cancer is a histologic diagnosis made based upon a vulvar biopsy.
Visual inspection of the vulva is performed based upon vulvar complaints or during a
routine pelvic examination. Findings that are suspicious for preinvasive or invasive vulvar
disease include any lesion that is raised or fungating as well as pigmented areas. If a
lesion is not grossly evident, but there is clinical suspicion of vulvar neoplasia, a five
percent acetic acid solution should be applied and the vulva examined with direct visual
inspection and using a colposcope (or a handheld magnifying glass if a colposcope is not
available). The concentrated acetic acid solution should be applied copiously with
prolonged contact to the keratinized vulvar squamous epithelium. This allows the cells to
fully dehydrate and will define acetowhite lesions and their underlying vascular changes.
Acetowhite areas with abnormal vascular patterns should be biopsied.
(See "Colposcopy".)
All areas of concern should be individually biopsied as cancer may be seen in areas with
multifocal vulvar intraepithelial neoplasia. The biopsy should be taken from the area(s) of
the lesion that appears most abnormal. If multiple abnormal areas are present, then
multiple biopsies should be taken to "map" all potential sites of vulvar pathology.
Pathologic evaluation may not be possible for specimens taken from areas of extensive
necrosis.

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