The skin of the head and neck is a common location for skin cancers to develop. The majority of skin cancers of the head and neck are basal cell and squamous cell carcinomas, with melanoma also occurring at a significant incidence. However, there are many other histologies that occur and raise diagnostic and treatment challenges. In this article, we review some of the more common histologies that are classified as rare skin malignancies of the head and neck. Specifically, epidemiology, pathogenesis, histopathology, and treatment options for each of these histologies will be discussed. There is a growing emphasis on Mohs micrographic surgery in the treatment of these lesions, although, some are not amenable to this technique. A multidisciplinary approach is frequently useful in their treatment.
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