Vulvar malignant melanoma: Pathogenesis, clinical behaviour and management: Review of the literature

•Vulvar malignant melanoma (VMM) is a distinct entity of mucosal melanoma with an unfavourable prognosis.•Surgery is the cornerstone of treatment for VMM.•There is no indication for lymph node dissection whilst the role of sentinel node biopsy is being studied.•There is an urgent need for new and better treatment strategies in VMM of which in particular immunotherapy is of high interest. Vulvar malignant melanoma (VMM) is a rare disease, accounting for 5% of all vulvar malignancies and is characterized by low survival and high recurrence rates. It is considered as a distinct entity of mucosal melanoma. Prognostic factors are higher age, advanced Breslow thickness, and lymph node involvement whilst central localization and ulceration status are still under debate. Surgery is the cornerstone for the treatment of primary VMM, however, it can be mutilating due to the anatomical location of the disease. Elective lymph node dissection is not part of standard care. The value of sentinel lymph node biopsy in VMM is still being studied. Radiation therapy and chemotherapy as adjuvant treatment do not benefit survival. Immunotherapy in cutaneous melanoma has shown promising results but clinical studies in VMM are scarce. In metastatic VMM, checkpoint inhibitors and in case of BRAF or KIT mutated metastatic VMM targeted therapy have shown clinical efficacy. In this review, we present an overview of clinical aspects, clinicopathological characteristics and its prognostic value and the latest view on (adjuvant) therapy and follow-up.