The complex management of atypical Spitz tumours

Abstract In recent years, advances in molecular genetic characterisation have revealed that atypical Spitz tumours (ASTs) are basically heterogeneous diseases, although the clinical relevance of these findings is yet to be determined. Evidence of molecularly-defined diverse groups of lesions continues to accumulate; however, conflicting, confusing, and overlapping terminology has fostered ambiguity and lack of clarity in the field in general. The lack of fundamental diagnostic (morphological) unambiguous classification framework results in a number of challenges in the interpretation of the molecular genetic data. In this review, we discuss the main difficulties for pathologists and clinicians in the complex management of ASTs, with particular emphasis on the different genetic and biological features of recently-described entities, and offer our view of what could be medically reasonable to guide a rational approach in light of current data.