Staging and treatment for vulvar cancer is surgical (

Table 49-1). The primary treatment for invasive vulvar cancer is complete surgical removal of all tumor whenever possible. The recent trend is toward a more conservative surgical approach, departing from traditional en bloc resections.

The number of preoperative studies ordered prior to surgery depends on the extent of disease and the general condition of the patient. A complete history and a thorough physical examination that includes cytologic study of the cervix and vulvoscopy should be performed. A large tumor may interfere with adequate pelvic examination.

Bleeding may be caused by a lesion higher in the genital tract rather than the obvious vulvar tumor. In that case, the pelvic examination may be performed under anesthesia, and endometrial biopsy or dilatation and curettage (D&C) considered.

Chest radiography, complete blood count, and urinalysis are performed on all patients. Older patients require an electrocardiogram (ECG) and a biochemical profile. Other studies such as proctoscopy, pyelography, barium enema, and computed tomography (CT) scans are ordered on an individual basis. Enlarged lymph nodes do not require biopsy; they will be excised by lymphadenectomy or thoroughly sampled at the time of operation. Mechanical bowel cleansing is recommended for most patients, particularly if the perineal skin is involved. An antibiotic bowel preparation is prescribed if extensive perianal dissection, skin grafting, or intestinal surgery (such as abdominoperineal resection) is anticipated. At least 2 units of packed red cells should be available for transfusion. Less than 50% of patients require a transfusion during or after the operation.

Historically, the basic operation was radical vulvectomy and regional lymphadenectomy. The trend, however, is shifting away from standard en bloc radical vulvectomy and bilateral lymph node dissection toward wide radical local excision of the primary tumor with inguinal lymph node dissection. For a unifocal stage I lesion with less than 1 mm stromal invasion, wide radical local excision with surgical margins of at least 1-2 cm should be performed. Patients with unilateral lesions with a depth of invasion greater than or equal to 1 mm should undergo ipsilateral groin dissection in addition to the above to determine nodal status. For patients with bilateral lesions or lesions impinging on or crossing the midline, bilateral inguinal femoral lymphadenectomy can be performed. When disease has spread to lymph nodes, adjuvant radiation therapy is generally recommended. The role of sentinel node mapping is also being evaluated for patients with squamous vulvar carcinoma and melanomas. In general, lymphatic spread occurs in a sequential manner from the superficial to the deep inguinal lymph nodes. Consequently, if the superficial nodes harbor no metastatic disease, there is reasonable assurance that the deeper nodes are not involved.

When the disease involves the anus, rectum, rectovaginal septum, proximal urethra, or bladder, an adequate surgical resection is only possible with pelvic exenteration combined with radical vulvectomy. Operative mortality is high for these procedures and the postoperative psychologic impact is significant. In addition, with advanced stage disease where ulcerated or fixed lymph nodes are palpated, attempts at lymphadenectomy have yielded very poor results. Based on data from the Gynecologic Oncology Group, this group of patients may benefit from preoperative chemoradiation resulting in higher rates of successful resection and reduced need for more radical surgery. Chemotherapeutic agents such as cisplatin and 5-FU have been combined with radiation therapy. These chemotherapeutic agents are used as radiation sensitizers in large necrotic tumor beds, enhancing the radiation effects.

There is controversy concerning the extent of surgery required for treatment of malignant melanoma of the vulva. For some years, standard treatment consisted of vulvectomy with superficial and deep inguinal and pelvic lymphadenectomy. It is also generally treated with a more conservative surgical approach. If depth of the vulvar lesion is less than 1 mm, vulvar melanoma may be adequately treated with local incision using a 1-cm margin. However, if the depth of invasion is between 1 and 4 mm, excision requires a 2-cm margin in addition to a bilateral groin node dissection. Advanced or recurrent melanoma may be best treated with chemotherapy, radiation, or immunotherapy.

Locally invasive but nonmetastasizing sarcomas such as dermatofibrosarcoma protuberans can be removed by wide local resection. Most other sarcomas are treated by radical vulvectomy and regional lymphadenectomy. The primary determinant of cure appears to be adequate wide removal of the primary lesion.