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Cancer of the Vulva

Invasive squamous cell carcinoma accounts for 90% of all invasive malignancies of the vulva, which are responsible for only 1-4% of all female cancers. Other less-common malignant lesions of the vulva are melanoma, adenocarcinoma, and sarcoma. More than three fourths of all patients diagnosed with cancer of the vulva are age 55 years or older, with at least 30% of these women older than 75 years. Approximately 500 deaths from vulvar cancer occur annually in the United States. Because of recognizable symptoms and ease of examination by biopsy, malignancies of the vulva can be detected in an early stage, when therapy can be curative.

The cause of vulvar malignancies remains unknown, although the association of squamous cell carcinoma of the vulva with other neoplasms of the anogenital mucosa has long suggested a common etiology. Preliminary data on oncogenesis, however, has not been conclusive. The association of high-risk types of human papillomavirus (HPV) such as 16, 18, 31, 33, 35, and 39 with high-grade epithelial neoplasia and invasive carcinomas of the anogenital tract has been established. Vulvar cancer appears to have a multifactorial etiology, however, and HPV infection alone.

Multicentric and confluent vulvar intraepithelial neoplasia (VIN) lesions predominate among younger women, whereas the unifocal lesions, which are most likely to be associated with invasive carcinoma, are more common in older women. The lesions may appear to be white because of thick surface keratin or red if hyperemia is present within the dermal papillae. Pigmentation is common, especially with bowenoid neoplasia.

The impetus to perform more conservative surgery for invasive cancer has been the realization that radical vulvectomy is associated with severe psychosocial sequelae. When compared with healthy adult women, women who have undergone a vulvectomy report lower levels of sexual arousal and poor body image.

Screening

No specific screening method is available. The profile of a vulnerable patient should always be considered. The average age at which invasive disease is diagnosed is 65 years. The most frequent symptom is a long history of vulvar itching, and the most common finding is a mass.

Carcinoma in Situ

Vulvar intraepithelial neoplasia has a peak onset in the fourth decade, preceding the most common age of onset of invasive carcinoma by 10-15 years. The incidence of vulvar carcinoma in situ has markedly increased, whereas the incidence of invasive cell carcinoma has remained stable. A possible explanation of this difference is that sexually active women are not yet old enough to have invasive vulvar carcinoma, and early diagnosis and treatment

Diagnosis

Vulvar intraepithelial neoplasia frequently is a multifocal disease; it most commonly affects the central vulva, with the lower half of the vulva most often affected. The mean thickness of the epithelium for all grades of VIN is 0.52 mm. The thickness of the involved epithelium varies with the location of the lesion. Infections caused by HPV are increasingly common and may be responsible for the increased frequency of the diagnosis of VIN. In the most overt form, HPV causes multiple condylomata that in themselves may have a significant degree of atypia and may be associated with intraepithelial neoplasia. Most subtle are the flat condylomata that may occur over the anogenital skin, producing irritative symptoms as well as atypical cytology and histology. Histologic examination of biopsies of specimens from such areas requires an experienced pathologist who is familiar with the subtleties of

Surgery

Because the biologic potential of VIN or carcinoma in situ remains uncertain, conservative therapeutic measures are appropriate. Previously, complete excision of the area at risk was recommended regardless of the size of the lesion. It is now recognized that these lesions are only part of the spectrum of neoplasias that occur over the entire anogenital epithelium. Removal of the entire area at risk may not be feasible and would certainly be disfiguring.

Laser therapy for ablation of an intraepithelial lesion of vulva may be appropriate in selected cases. One of its major limitations is the loss of tissue for histologic interpretation to detect occult invasion. Laser vaporization to a depth of 1 mm including the zone of dermal necrosis should be sufficient to

Invasive Squamous Cell Carcinoma

Invasive squamous cell carcinoma of the vulva accounts for approximately 5% of all genital malignancies and 90% of vulvar malignancies. The disease occurs primarily in the sixth and seventh decades of life, although women age 25 years or younger can have invasive squamous cell carcinoma of the

Vulvar cancer spreads by direct extension to the adjacent structures, including the vagina and anus, and by lymphatic embolization to the regional lymph nodes. Metastases to the liver, lungs, and bones may occur. The overall incidence of lymph node metastasis of vulvar cancer is about 30%. When inguinal-femoral node metastasis is present, the rate of pelvic node metastasis is about 25%.

The incidence of groin node metastasis is related to the depth of stromal invasion, grade of the tumor, presence of lymphovascular space involvement, clinical node assessment, and age of the patient.

Lesions smaller than 2 cm in diameter with minimal stromal invasion of less than 1 mm have been designated stage IA. If these lesions have no

Treatment

Significant advances have occurred in the management of squamous cell carcinoma of the vulva with the advent of conservation for unifocal lesions and limitation of pelvic lymphadenectomy. Now, full groin dissection can be avoided in some patients with stage IA vulvar carcinoma. In stage IB, contralateral groin dissection in patients with lesions smaller than 2 cm and negative ipsilateral nodes has been eliminated. Also, separate groin incisions

The primary morbidities of radical vulvectomy and bilateral groin dissection are lymphedema and groin wound breakdown. Few intraoperative deaths occur, but there is a postoperative mortality rate of 5% because of the geriatric population affected. Radical vulvectomy is often complicated by problems associated with insufficient closure of a large skin defect contributing to postoperative necrosis of the suture line over the mons pubis and the

Radiation therapy with megavoltage equipment using electron beam has been applied as primary treatment of extensive carcinoma of the vulva. In

Modified from International Federation of Gynecology and Obstetrics. Annual report on the results of treatment in gynecological cancer. 22nd edition. Stockholm: FIGO

Prognostic Factors

The 5-year survival rate for operable cases is about 70%. Patients with negative groin Nodes have a 5-year survival rate of 90%, but this falls to about 50% for patients with positive Nodes. The number of positive Nodes is the single most important prognostic variable, and patients with one

Paget's Disease

Paget's disease of the vulva has a high recurrence and a higher incidence of invasive disease than was previously recognized. The median age of diagnosis is 64 years. About 10-15% of patients have been reported to have an associated invasive adenocarcinoma of the vulva at the time of

Paget's disease of the vulva occurs most often in white postmenopausal women presenting with extreme pruritus and soreness, often of long duration. Disease appears as red or bright pink, desquamated, eczematoid areas among which are scattered, raised, white areas of hyperkeratosis. The borders appear slightly elevated and sharply demarcated. Perianal involvement is Not uncommon, but other areas of the aNogenital tract, cervix, and vagina

Verrucous Carcinoma

Verrucous carcinoma of the vulva is much less common than squamous cell carcinoma. The clinical and morphologic distinctions between these neoplasms are important to understand because of their contrasting biologic behavior and treatment. Verrucous lesions rarely metastasize.

Both cancers present with symptoms of pruritus and a Noticeable mass. On examination, both tumors commonly occur on the labia and are exophytic. If infection occurs in association with verrucous carcinoma, the resulting induration of the surrounding tissue as well as reactive regional lymph Node enlargement may lead to an erroneous diagnosis of advanced squamous cell carcinoma. One third of squamous cell carcinomas are flat and ulcerative.

Melanomas

Melanomas are the second most common vulvar malignancy and occur frequently in white women between the ages of 50 and 80 years. Melanomas may arise from preexisting lesions and occur mainly on the labia minora and clitoris. Patients at increased risk for melanoma include those with a family history of melanoma and those with dysplastic nevi elsewhere on the body. Dysplastic melanoma nevi occur in 2-5% of white adults and in one third of patients with cutaneous melanoma. Most patients present with a lump or tumor on the external genitalia, although complaints of itching, bleeding, or

Bartholin Gland Cancer

Bartholin gland cancer is a rare malignancy that should be considered in the differential diagnosis of a labial mass. The tentative diagnosis of adenocarcinoma of the Bartholin gland is based on the cytologic findings and location of the tumor. The nuclei are oval to oblong, and some cells have a peripherally displaced nucleus. The chromatinic material is slightly increased, and some nuclei have prominent nucleoli. The cytoplasm is basophilic

Sarcoma

Soft tissue sarcomas make up fewer than 2% of vulvar malignancies. They occur over a wide age range, including the pediatric population, and usually appear as a rapidly enlarging and painful mass. Most tumors on the vulva are related to the leiomyosarcoma group, although the fibrous histiocytomas,

Basal Cell Carcinoma

Among the rarest of the vulvar carcinomas, basal cell carcinomas occur once for every 40 invasive squamous cell carcinomas. They are distinguished by cords and masses of palisading basal cells pushing into the underlying connective tissue, and, like basal cell carcinomas elsewhere, they do Not metastasize. A history of longstanding vulvar pruritus and delay in diagnosis are common. The lesions frequently have a slightly elevated margin at their periphery. Basal cell carcinomas are most commonly found over the anterior two thirds of the labia majora and occur most frequently in white women