ABSTRACT
Atypical spitzoid tumors are a morphologically diverse group of rare melanocytic lesions most frequently seen in children and young adults. They can be included in an intermediate group that can mimic both Spitz nevus, with a benign course, and spitzoid melanoma, with a malignant potential: for this reason, it is crucial to recognize these entities clinically and histopathologically even if their real nature is still a cause of debate among dermatopathologists. Since the first report in the literature, several studies have been done on their histopathologic features without reaching a consensus on their behavior. In recent years, some authors proposed a diagnostic algorithm for atypical spitzoid tumors by using a combination of immunohistochemical and cytogenetic/molecular tests; others described their clinical and dermoscopic features to better recognize them. The role of sentinel lymph node biopsy is debated, even if systematic review did not show any prognostic benefit of this procedure.
