Radical vulvectomy with inguinal and femoral lymphadenectomy is the standard therapy. The definition of radical vulvectomy is being extended with the realization that the effect of radical surgery is limited by the closest resection margin, rather than the achievement of total organ ablation.[1] Nodal involvement is a key determinant of survival. The 5-year survival rate for patients with unilateral nodal involvement is 70%, with a decrease to 30% for those with three or more unilateral nodes involved.[2]

In a randomized trial from the Gynecologic Oncology Group, patients with two or more pathologically positive groin nodes had significantly better survival with radiation therapy to the groin and pelvis than with pelvic node dissection. Patients on both arms of the trial received radical vulvectomy and bilateral inguinal and femoral groin node dissections. Patterns of failure have shown a significant decrease in groin failures with radiation therapy to the groin and pelvis compared with pelvic node dissection.[3]

STANDARD TREATMENT OPTIONS:

1. Modified radical vulvectomy with inguinal and femoral node dissection. Radiation therapy to the pelvis and groin should be performed if inguinal nodes are positive.
2. Radical vulvectomy with inguinal and femoral node dissection followed by radiation therapy to the vulva in patients with large primary lesions and narrow margins. Localized adjuvant radiation therapy consisting of 45 Gy to 50 Gy may also be indicated when there is capillary-lymphatic space invasion and a thickness of greater than 5 mm, particularly if the nodes are involved.[1] Radiation therapy to the pelvis and groin should be performed if two or more groin nodes are involved.[3]
3. Preoperative radiation therapy may be used in selected cases to improve operability and even decrease the extent of surgery required.[4,5] A radiation dose of up to 55 Gy with concomitant fluorouracil (5-FU) has been suggested.[1]
4. For those patients unable to tolerate radical vulvectomy or who are deemed unsuitable for surgery because of site or extent of disease, radical radiation therapy may result in long-term survival.[6,7] Where radiation therapy is being tested for primary definitive treatment of vulvar cancer, some prefer to add concurrent 5-FU or 5-FU and cisplatin.[1,8,9,10,11] Four phase II trials of concurrent 5-FU with or without cisplatin with radiation resulted in complete response rates of 53% to 89% for primary unresectable disease or for those who would require exenterative surgery.[8,9,10,11] With a median follow-up of 37 months, two series report crude disease-free survival rates of 47% to 84%.[9,10] Radiation complications of late fibrosis, atrophy, telangiectasia, and necrosis are minimized if the radiation fraction size is less than or equal to 1.8 Gy and excessive total doses are not used.[1,8,9,10,11] Doses of at least 54 Gy but less than 65 Gy should be used.