Management of vulvar and vaginal melanomas: current and future strategies.

Melanomas arising in the vulva and vagina are rare and therefore there is minimal data specific to these malignancies. Data are often extrapolated from other cutaneous melanomas, which may or may not be appropriate. Surgery remains the primary treatment modality at initial diagnosis and in select recurrent cases. Wide local excision of the primary lesion not requiring an exenteration, along with sentinel lymph node (SLN) mapping, should be routinely considered in vulvar melanomas. Local excision and SLN mapping is difficult and often not considered for vaginal melanomas. Primary exenterative procedures should not be routinely offered. In locally advanced cases potentially requiring an exenterative procedure, radiation therapy with or without concurrent immunotherapy is a consideration. The role of adjuvant therapy remains unclear. Surgery or radiation therapy can be considered in recurrent cases. Systemic chemotherapy agents have a modest response rate with associated poor survival outcomes. Novel immunotherapeutic and targeted agents have been reported to improve survival in melanoma and should be considered in cases of vulvovaginal melanoma. All cases should be tested for at least c-KIT and BRAF V600E mutations. Patients with vulvovaginal melanomas should be strongly encouraged to participate in clinical trials.