Vulvar Cancer Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Option Overview
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.
Metastases at diagnosis are detected in approximately 25% of patients. The prognosis of patients with metastatic disease is poor. Current therapies for patients who present with metastatic disease achieve 6-year event-free survival (EFS) of approximately 28% and overall survival (OS) of approximately 30%.[2,3] For patients with lung/pleural metastases only, 6-year EFS is approximately 40% when utilizing bilateral lung irradiation.[2,4] In contrast, patients with bone/bone marrow metastases have...
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).
In a multicenter case series, 403 patients with primary vulvar squamous cell cancers smaller than 4 cm and clinically negative groin nodes underwent 623 sentinel node dissections using radioactive tracer and blue dye for sentinel node identification. All patients had radical resection of the primary tumor. Node metastases were identified in 26% of sentinel node procedures, and these patients went on to full inguinofemoral lymphadenectomy. The patients with negative sentinel nodes were followed with no further therapy.
Local morbidity was much lower in patients who underwent sentinel node dissection than in patients with positive sentinel nodes who also underwent inguinofemoral lymphadenectomy: wound breakdown 11.7% vs. 34.0%; cellulitis 4.5% vs. 21.3%; chronic lymphedema 1.9% vs. 25.2%, respectively (P < .0001 for all comparisons). Mean hospital stay was also shorter: 8.4 vs. 13.7 days (P < .0001). After two local recurrences in 17 patients with multifocal primary tumors, the protocol was amended to only allow patients with unifocal tumors into the study. Actuarial groin recurrence for all patients with negative sentinel node dissections at 2 years was 3% (95% confidence interval [CI], 1%–6%) and 2% (95% CI, 1%–5%) for those with unifocal primary tumors.[Level of evidence: 3iiiDiv]