Three distinct patterns of local recurrence were described: remote vulvar recurrence (greater than 2 cm of the primary tumor site), primary tumor site recurrence (within 2 cm of the primary tumor site), and skin bridge recurrence (81,150). Although reported treatment rates are excellent for remote site recurrences with 3-year survival rates of 67% to 100%, the literature is controversial as to the prognostic significance of primary tumor site recurrences, with one study reporting a 3-year survival rate of only 15% and the other a 5-year survival of 93% (81,150). Patients with skin bridge recurrence have a very poor prognosis, similar to those with groin recurrence.
Regional and Distant Recurrence Regional and distant recurrences are difficult to manage and are associated with a poor prognosis (141,145,146). Radiation therapy may be used in conjunction with surgery for groin recurrence, whereas chemotherapeutic agents that have activity against squamous carcinomas may be offered for distant metastases. The literature on the use of chemotherapy for recurrent vulvar cancer consists mainly of small series. The most extensively studied regimens contain bleomycin, methotrexate, and lomustine (a nitrosourea); bleomycin and mitomycin C; or cisplatin, vinorelbine, and paclitaxel, but response rates are low and the duration of response is usually disappointing (153–157). Long-term survival is very uncommon with regional or distant recurrence. Symptom control and quality of life are important treatment goals, and early involvement of a multidisciplinary palliative care team is generally indicated.
Vulvar melanomas are rare, with an incidence of 0.1 to 0.19 per 100,000 women (158,159). They account for 4% to 10% of all cases and are the second most common form of vulvar malignancy. Most melanomas arise de novo, but they may arise from a preexisting junctional nevus (160). Vulvar melanomas occur most frequently in postmenopausal white women, but are commonly seen in individuals with darker pigmented skin. Contrary to cutaneous melanoma, based on SEER data, there are low racial differences in vulvar melanomas, the development of which appears to be determined by factors other than ultraviolet radiation exposure and the photoprotective effects of melanin seen in cutaneous melanoma (161). The incidence of cutaneous melanomas worldwide is increasing significantly, but not that of vulvar melanoma (161). Vulvar melanomas appear to behave in a manner similar to that of other truncal cutaneous melanomas (162–165).
Most patients with vulvar melanoma have no symptoms other than a pigmented lesion that may be enlarging. Some patients have itching or bleeding, and a few have a groin mass. Vulvar melanomas occur most frequently on the labia minora or the clitoris (Fig. 38.6), and extension into the urethra or vagina at discovery is not uncommon. Any pigmented lesion on the vulva should be excised or, if the lesion is large, sampled for biopsy unless it is known to have been present and unchanged for some years. Most vulvar nevi are junctional and may be precursor lesions to melanoma; any nevus of the vulva should be removed.
Figure 38.6 Melanoma of the vulva involving the right labium minus. (From Berek JS, Hacker NF. Berek & Hacker’s Gynecologic Oncology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2010:564, with permission.)
Figure 38.7 Vulvar melanoma. Spindle-shaped melanoma cells form interlacing bundles, and some contain melanin pigment (right upper corner). Epidermal invasion is evident in the form of Pagetoid migration (left upper corner).
Table 38.7 Microstaging of Vulvar Melanoma