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Vulvar Cancer

Cancer of the Vulva

Vulvar Cancer

Preinvasive Disease of the Vulva

Extramammary Paget's Disease

Vulvar cancer is an uncommon malignancy, responsible for only 5% of all gynecologic neoplasms, but its incidence is on the rise. Although the age of onset of vulvar cancer varies widely, most cases occur in women over age 50. Peak incidence occurs between the ages of 65to 70.

Epidemiologic studies have shown associations between vulvar cancer and genital warts, abnormal Pap smears, and smoking. Molecular analysis of vulvar neoplasias has revealed the presence of human papillomavirus (HPV) genomic material. These findings support an etiologic relationship between papillomavirus and vulvar cancer. Vulvar intraepithelial neoplasia (VIN) predisposes to vulvar cancer. Lichen sclerosis is not generally considered to be a premalignant condition. Squamous cell hyperplasia is often found in regions adjacent to vulvar cancer, but the relationship of this lesion to invasive cancer is unclear. Advanced age and race (white) have been shown to be independent predictors of poor outcome.

Although it seems intuitive that vulvar cancer would be relatively easy to diagnose because of the accessibility of the vulva to visual inspection, clinical findings may be subtle. Most patients experience a delay in diagnosis; this happens for a variety of reasons. Women may dismiss symptoms as mild or unimportant and delay seeking medical attention.

Alternatively, many physicians treat patients on the basis of their symptoms without performing a biopsy of the lesion. About 50% of vulvar cancer patients experience prolonged pruritis or have an ulcerated or palpable mass on their vulva.

Vulvar cancer is easily mistaken for other conditions.

There is no absolute consistency in the appearance of vulvar lesions such as VIN, lichen sclerosis, or squamous cell hyperplasia that allows them to be easily distinguished from vulvar cancer. Therefore, it is best to advise that any suspicious areas should be biopsied. Examination of the vulva should include a thorough inspection of the perineal area, including areas around the clitoris and urethra. Palpation of the Bartholin's glands should be performed as well. Biopsies may be guided by use of toluidine blue dye (1%) or dilute acetic acid. Uptake of blue dye is increased in neoplastic cells with enlarged nuclei. This increased nuclear to cytoplasmic ratio is also responsible for the altered appearance of neoplastic cells treated with acetic acid (causing them to appear more white than surrounding tissues). Abnormally pigmented lesions suspicious for melanoma should be biopsied by a gynecologist or gynecologic oncologist familiar with the anatomy of the region because wide excisional margins are required.

More than 85% of vulvar malignancies are of squamous histology. About 5% of vulvar cancers are melanomas. Squamous cell cancer of the vulva is usually indolent, growing slowly and metastasizing late in its course. Local extension of the tumor occurs first, followed by lymphatic invasion. In general, lymphatic spread occurs in an organized, progressive fashion with superficial inguinal nodes affected before deeper (inguinofemoral and pelvic) nodal chains become involved.

Staging and therapy are primarily surgical, and the extent of resection is related to the stage of the tumor and the likelihood of nodal metastases. For lesions with less than 1 mm of invasion, radical wide local excision will suffice. Stage I lesions (≤2 cm) can be treated effectively with modified radical hemivulvectomy and ipsilateral superficial inguinal node dissection (Figure 37.6). Larger tumors usually require radical vulvectomy and inguinal lymphadectomy (Figure 37.7). Larger tumors may also be treated primarily with radiotherapy and concurrent chemotherapy followed by surgical resection of residual disease. Pelvic exenteration is sometimes employed for women with extensive disease or as a salvage strategy for patients with recurrent cancer. Postoperative morbidity is related to the extent of the surgery. Therefore, the most conservative operation that can effectively treat the patient's condition is performed. As with the cancers previously discussed here, elderly patients with vulvar cancer should be allowed to receive the full benefit of optimal surgical therapy, as age alone is a poor determinant of surgical risk.

Survival is related to clinical stage and the extent of nodal involvement. Five-year survival for stages I and II vulvar cancer treated by standard surgical means is of the order of 90%. Survival (for all stages) still exceeds 70% in the absence of lymph node metastasis. However, lymphatic spread portends a worse outcome. Patients found to have inguinal lymph node metastases are usually treated with inguinal and pelvic radiation therapy postoperatively.

Gynecologic Cancers

Only one-third of patients survive 5 years if nodal metastasis is present. Involvement of the deeper, pelvic nodes results in a 20% 5-year survival rate. Approximately 80% of recurrences occur in the first 2 years after treatment. The majority of recurrences occur near the site of the primary lesion. Seventy-five percent of patients with locally recurrent disease (limited to the vulva) can be salvaged with radical wide local excision. In contrast, patients who develop a groin recurrence are rarely curable, and palliative surgical resection is associated with a high risk of complications.

The most common complications of treatment are related to wound breakdown and infection and occur in approximately 15% of patients. Other complications that arise in the early postoperative period include urinary tract infection, hematomas, seromas, venous thromboembolic disorders, femoral nerve injury manifestations, and osteitis pubis. Late complications may include lymphangitis, stress urinary incontinence, lymphocyst formation, introital stenosis, vaginal fistula, femoral hernia, and sexual dysfunction, but the most common late complication is intractable leg edema (lymphedema).

Estrogen replacement can usually be given to these patients. Hormonal treatment may help to maintain the health of surrounding perineal and vaginal tissues postoperatively.

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