Standard primary treatment for vulvar cancer is surgery. Radiation is usually added to surgery in patients with stage III or IV disease.[1,2,3] Newer strategies have integrated surgery, radiation therapy, and chemotherapy and tailor the treatment to the extent of clinical and pathologic disease. Patterns of practice in combining these treatments vary.
Since invasive and preinvasive neoplasms of the vulva may be HPV-induced and the carcinogenic effect may be widespread in the vulvar epithelium, patients should be followed regularly for symptoms or signs of recurrence. Because there are few patients with advanced disease (stages III and IV), only limited data are available on treatment efficacy in this setting, and there is no standard chemotherapy regimen for these patients. Physicians should offer eligible patients with stage III or IV disease participation in clinical trials.
Incidence and Mortality
Vulvar cancer accounts for about 5% of cancers of the female genital system in the United States.
Estimated new cases and deaths from vulvar cancer in the United States in 2014:
New cases: 4,850.
The vulva is the area immediately external to the vagina, including the mons pubis, labia, clitoris, Bartholin glands, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases...
Information about ongoing clinical trials is available on the NCI Web site.
Role of Surgery
Until the 1980s, the standard therapeutic approach to therapy for invasive locoregional vulvar carcinomas was radical surgery, including complete en bloc resection of the vulva and regional lymph nodes. Because of the high attendant complication rates, wound healing problems, lymphedema, and functional deficits, the trend since then has been toward more limited surgery, often combined with radiation therapy. (Refer to the Role of Radiation Therapy section of this summary for more information.)
In tumors clinically confined to the vulva or perineum, radical local excision with a margin of at least 1 cm has generally replaced radical vulvectomy; separate incision has replaced en bloc inguinal node dissection; ipsilateral inguinal node dissection has replaced bilateral dissection for laterally localized tumors; and femoral lymph node dissection has been omitted in many cases. However, the different surgical techniques have not been directly compared in randomized controlled trials. In addition, even the nonrandomized studies suffer from lack of uniform staging definitions and clear descriptions of lymph node dissection or ancillary radiation.[Levels of evidence: 3iiiDii, 3iiiDiv] The evidence base is therefore limited.
Another strategy to minimize the morbidity incurred by groin-node dissection in patients with early clinical-stage disease is sentinel node dissection, reserving groin dissection for those with metastases to the sentinel node(s).