Mucosal Melanoma

Abstract

Mucosal melanomas are rare tumors which most commonly arise in the upper aerodigestive tract (oral cavity, nasal cavity, and sinuses), anorectum, and female genital tract. Compared to cutaneous melanomas, far less is known about the pathogenesis, natural history, and management of mucosal melanomas. At presentation, mucosal melanomas are characteristically more advanced and associated with poorer outcomes than cutaneous melanomas. The primary treatment modality is complete surgical excision, but due to the anatomical location and advanced stage at presentation, complete removal may not be possible. As the overwhelming majority of patients with locally advanced tumors will die of metastatic disease, highly morbid and/or extensive procedures with major impact on quality of life may not be justified. For patients with advanced disease, immunotherapy with anti PD-1 therapy should be considered and similarly for the small proportion of patients with a c-kit mutation targeted therapy with imatinib may be worthwhile. Unfortunately, the results of treatment for advanced disease do not match those seen for cutaneous melanoma. In this chapter, we review the clinical and pathologic features of mucosal melanomas in general and provide a more detailed discussion concerning the presentation and management of tumors originating in specific anatomical locations. Management of advanced disease is considered separately.