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Melanoma pp 1–14Cite as

Mucosal Melanoma

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Abstract

Mucosal melanoma (MM) is a distinct entity with unique pathologic features and clinical behavior. It is a rare entity accounting for only 1–2% of all melanoma cases. Head and neck region mucosa comprises the majority of MMs. The nose and paranasal sinuses are by far the most common site representing 59–80% of all Head and Neck MM and oral cavity tumors representing the majority of the remaining cases. The pathogenesis of these tumors is poorly understood. Premalignant pigmented mucosal lesions have been identified and may evolve similar to cutaneous melanoma. Presentation of MM varies greatly depending on the location of the primary. Because a lesion may remain undetected for long periods of time in the nasal cavity, for example, MM commonly presents at an advanced stage. However, even patients who initially present with small localized disease have a high risk of distant relapse making survival rates for MM very low. The mainstay of treatment is surgical removal of the primary melanoma, which is not always possible in certain locations. While adjuvant radiation is often considered, definitive data showing clinical benefit is lacking. Recent progress in immunotherapy for cutaneous melanoma has yet to be fully assessed and understood for MM. Mucosal melanomas have distinct genetic alterations, including activating mutations of KIT, which offer an opportunity for targeted therapy in a subset of cases.

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Correspondence to Tjoson Tjoa .

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Tjoa, T., Emerick, K. (2018). Mucosal Melanoma. In: Fisher, D., Bastian, B. (eds) Melanoma. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-7322-0_4-1

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