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Most patients with SM, particularly those who present with aggressive clinical courses, carry somatic mutations of the v\u2010kit Hardy\u2010Zuckerman 4 feline sarcoma viral oncogene homolog (<jats:italic>KIT<\/jats:italic>) gene. KIT mutations are considered central events in the pathogenesis of SM and serve as diagnostic markers and putative therapeutic targets. The heterogeneity in the clinical course of patients with SM and recent advances in the genetic and immunophenotypic characterization of neoplastic mast cells may help to improve current diagnostic, taxonomic, and therapeutic approaches in SM. 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