Large tumors of the vulva encroaching on the anorectal area and the urethra require more extensive treatment than a radical vulvectomy. In addition to a radical vulvectomy, it is usually necessary to perform a diversion of the urinary or fecal stream. If the nodes are negative, a 5-year survival rate of 50% has been reported.

However, newer approaches that involve a combination of preoperative radiation and surgery have been reported in treating these lesions. External radiation often is given to reduce the size of the tumor before surgical removal by radical vulvectomy, with or without regional lymph node dissection.

Approximately 4,000 to 4,500 cGy is delivered to the pelvis and inguinal nodes, the operation being performed 5 weeks after the completion of radiation. This approach may obviate a urinary or fecal diversion. Boronow and colleagues reported a 5-year survival rate of 80% in 26 patients with primary carcinoma of the vagina and vulva who were treated with this technique. Rotmensch and colleagues recently reported on 16 patients with advanced vulvar lesions who were treated with preoperative radiation to the vulva and achieved an overall 5-year survival rate of 45%. Recurrences were more likely if the resection margins were within 1 cm of the tumor. Complications have included stenosis of the introitus and urethra as well as rectovaginal fistula.

Recently, an approach has been to not only administer preoperative radiation for advanced lesions but also to add chemotherapy. Commonly, agents such as 5-FU, cisplatin, and mitomycin C have been used, producing up to 46% reduction in tumor with chemoradiation. The most common toxicities were acute cutaneous and wound complications. Investigators have suggested that radiation-sensitive chemotherapeutic drugs, such as cis-platinum, may enhance the response to radiation. The GOG has attempted to expedite this; however, the study was terminated owing to a lack of sufficient patients.

Because the vulvar skin is prone to radiation dermatitis, fibrosis, and ulceration, radiation as the sole therapy has been less than desirable. If the patient is inoperable because of medical conditions, however, radiation can be used as the primary treatment of a vulvar carcinoma. Recurrences may be local or distant. More than 80% of recurrences occur in the first 2 years after therapy. The risk of recurrence of vulva carcinoma increases as the stage of disease increases. In an analysis of 224 patients with vulvar carcinoma, Podratz and colleagues reported a recurrence rate of 14% in stage I and 71% in stage IV disease. Local recurrences were the most common. Local recurrences often occur in the skin bridge remaining after a radical vulvectomy.

Different modalities have been used to treat local recurrences. Both radiation therapy and resection of local vulvar recurrence provide effective control and a 5-year survival rate of approximately 50%.

The combination of chemotherapy and radiation therapy has been used to treat recurrent disease and some large primary vulvar carcinomas. Thomas and colleagues reported the use of 5-FU (1 g/m2) as a continuous intravenous infusion for 4 to 5 days every 4 weeks during radiation. This combined approach was the sole treatment in nine patients, and six patients had a complete remission. Disseminated disease requires chemotherapy, but, unfortunately, no chemotherapy has been successful in this situation.