An uncommon cancer of women in which the malignancy
is in the vulva, the outer part of the vagina, that includes the labia. Cancer of
the vulva occurs mainly in women over 50 although it is becoming more common in
younger women. Clues may include constant itching, severe burning or pain, whitening
or roughening of the skin of the vulva, and bleeding or discharge that is not related
to menstrual periods.
Vulvar cancer is highly curable when diagnosed in an early stage. The chance
of survival is most dependent on the status of the inguinal lymph nodes, those in
the groin. Without nodal involvement, the overall 5-year survival rate is 90%. However,
with nodal involvement, the overall survival rate drops to about 50 to 60%. Survival
is dependent to a lesser degree on the diameter of the primary lesion (where the
cancer of the vulva arose). If that diameter is less than 2 cm, it makes for a better
prognosis (outlook).
The most common site of the cancer is the labia majora (about 50% of cases).
The labia minora accounts for 15% to 20% of cases. The clitoris and Bartholin's
glands are less frequently involved. Vulvar cancer is most commonly squamous cell
carcinoma in type, although other types do occur. In many cases, the development
of vulvar cancer is preceded by condyloma or squamous dysplasias. The human papillomavirus
(HPV) is believed to be a causative factor and to induce many of these tumors.
The pattern of spread of the tumor is influenced by the histology. Well-differentiated
lesions are less malignant and tend to spread along the surface with minimal invasion,
while anaplastic ("wild-looking") tumors are more likely to be deeply invasive.
Spread beyond the vulva is either to adjacent organs such as the vagina, urethra,
and anus, or via the lymphatics to the regional lymph nodes. Spread of the tumor
though the bloodstream is uncommon.
The standard treatment for vulvar cancer diagnosed at an early stage is usually
surgery. For most patients with more advanced disease, the standard treatment is
generally surgery supplemented by external beam radiation therapy. Newer strategies
may integrate the possible therapeutic advantages of surgery, radiation therapy,
and chemotherapy and tailor the treatment to the extent of the disease in the individual
woman. Because of the psychosexual consequences and significant disease associated
with standard radical vulvectomy, there is a definite trend toward vulvar conservation
and individualized management of patients with early vulvar cancer.
Since invasive and preinvasive neoplasms of the vulva may be HPV-induced and
the carcinogenic effect may be widespread in the vulva, close follow-up of patients
is mandatory so that early detection of recurrent or second tumors is possible.
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