The authors review the epidemiologic, clinicopathologic, and molecular features of anorectal melanoma, and discuss the differences between this uncommon and lethal disease and the more common and curable cutaneous form. Observations regarding outcomes after surgical resection will also be discussed, as well as predictors of survival and the use of adjuvant therapies. The recent discovery that the KIT oncogene may be aberrantly activated in a subset of patients with anorectal melanoma creates a realm of possibility for the development of targeted molecular therapy. Melanoma of the anorectum is an extremely rare tumor that is often difficult to diagnose. Its surgical management is controversial, and the prognosis remains poor.
