Clinical Features

Itching is the most common symptom, although some patients present with palpable or visible abnormalities of the vulva. About half of patients are asymptomatic. There is no absolutely diagnostic appearance. Most lesions are elevated, but the color may be white, red, pink, gray, or brown (Figure 40-1). About 20% of the lesions have a “warty” appearance, and the lesions are multicentric in about two thirds of cases.

FIGURE 40-1 Vulvar intraepithelial neoplasia type III: carcinoma in situ of the vulva. Note the pigmented and multicentric nature of the lesions and the extensive perianal involvement in this patient.

Diagnosis

Careful inspection of the vulva in a bright light, with the aid of a magnifying glass if necessary, is the most useful technique for detecting abnormal areas. Colposcopic examination of the entire vulva after the application of 5% acetic acid will sometimes highlight additional acetowhite areas.

Management

The mainstay of treatment is local superficial surgical excision, with primary closure. The microscopic disease seldom extends significantly beyond the colposcopic lesion, so margins of about 5 mm are usually adequate. For extensive lesions involving most of the vulva, a “skinning” vulvectomy, in which the vulvar skin is removed and replaced by a split-thickness skin graft, may be used. Because the subcutaneous tissues are not excised, the cosmetic result is superior to that obtained with vulvectomy.

Laser therapy is also effective, particularly for multiple small lesions, or for lesions involving the clitoris, labia minora, or perianal area. No specimen is available for histologic study after laser ablation, so a liberal number of biopsies must be taken before treatment to exclude invasive cancer.

Clinical Features

Itching and tenderness are common and may be long-standing. The affected area is usually well demarcated and eczematoid in appearance, with the presence of white plaquelike lesions. As growth progresses, extension beyond the vulva to the mons pubis, thighs, and buttocks may occur; rarely, it may extend to involve the mucosa of the rectum, vagina, or urinary tract. In 10% to 20% of cases, Paget’s disease is associated with an underlying adenocarcinoma.

Histologic Features

The disease is an adenocarcinoma in situ and is characterized by large, pale, pathognomonic Paget’s cells, which are seen within the epidermis and skin adnexa. They are rich in mucopolysaccharide, a diastase-resistant substance that stains positive with periodic acid–Schiff stain. The intracytoplasmic mucin may also be demonstrated by Mayer’s mucicarmine stain. The Paget’s cells are typically located adjacent to the basal layer, both in the epidermis and in the adnexal structures.

Management

The histologic extent of Paget’s disease is frequently far beyond the visible lesion. Local superficial excision with 5- to 10-mm margins is required to clear the gross lesion, exclude underlying invasive cancer, and relieve symptoms. Recurrences are common and may be treated by further excision or laser therapy. If an underlying invasive carcinoma is present, the treatment should be the same as for other invasive vulvar cancers.

Clinical Features

Patients generally present with a vulvar lump, although long-standing pruritus is common. The lesions may be raised, ulcerated, pigmented, or warty in appearance, and definitive diagnosis requires biopsy under local anesthesia. Most lesions occur on the labia majora; the labia minora are the next most common sites. Less commonly, the clitoris or the perineum is involved (Figure 40-2). About 5% of cases are multifocal.

FIGURE 40-2 A small perineal carcinoma. Note that the remainder of the vulva is normal.

Methods of Spread

Vulvar cancer spreads by direct extension to adjacent structures, such as the vagina, urethra, and anus; by lymphatic embolization to regional lymph nodes; and by hematogenous spread to distant sites, including the lungs, liver, and bone. In most cases, the initial lymphatic metastases are to the inguinal lymph nodes, located between Camper’s fascia and the fascia lata. From these superficial nodes, spread occurs to the femoral nodes located medial to the femoral vein. Cloquet’s node, which is situated beneath the inguinal ligament, is the most cephalad of the femoral node group. From the inguinofemoral nodes, spread occurs to the pelvic nodes, particularly the external iliac group (Figure 40-3).

FIGURE 40-3 Lymphatic drainage of the vulva. Inguinal nodes are displayed on the right side of the groin, and femoral nodes are seen on the left side of the groin.

The incidence of lymph node metastases in vulvar cancer is about 30%. It is related to the size of the lesion (Table 40-1). About 5% of patients have metastases to pelvic lymph nodes. Such patients usually have three or more positive unilateral inguinofemoral lymph nodes. Hematogenous spread usually occurs late in the disease and rarely occurs in the absence of lymphatic metastases.

TABLE 40-1 INCIDENCE OF LYMPH NODE METASTASES IN RELATION TO LESION SIZE IN VULVAR CANCER

Staging

In 1989, the International Federation of Gynecology and Obstetrics (FIGO) Cancer Committee introduced a surgical staging system for vulvar cancer. This system was revised in 1994, and the present FIGO staging system is shown in Table 40-2.

TABLE 40-2 INTERNATIONAL FEDERATION OF GYNECOLOGY AND OBSTETRICS (FIGO) STAGING OF VULVAR CARCINOMA (1994)

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