A classification of spitzoid melanocytic lesions into tumors (without or with atypical features) and melanoma has been recently proposed, thereby underlining the existence of a morphobiologic spectrum of lesions, ranging from benignity to full-blown malignancy. However, only in rare instances are spitzoid lesions clinically tumors (ie, cutaneous elevations [nodules] exceeding the size of a cherry [>2 cm]).

Dermoscopy has demonstrated that the pigmented (brown-black) variant of Spitz nevus is more common than the classical (pink-red) variant; the main dermoscopic patterns are defined as globular, starburst, and multicomponent (atypical, melanoma-like). In clinicopathologic studies the classical (pink-red) variant of Spitz nevus, dermoscopically typified by dotted vessels and white network, is surprisingly rare and is an atypical Spitz nevus or a Spitz tumor with a greater probability than a brown-black plaque.

A grading system for atypical spitzoid lesions is desirable to avoid overtreatment, mainly in prepubescent patients. We propose a distinction between atypical Spitz nevus and (atypical) Spitz tumor, based on the presence of tumorigenic features (ulceration, dermal sheets of cells, numerous, or deep-sited mitoses) solely in the latter.

Pediatric spitzoid melanoma is genetically different from its adult counterpart and has very peculiar clinicopathologic features, namely the presence of an overtly malignant nonspitzoid morphologic clone arising in the context of a spitzoid lesion. Such a highly unusual occurrence can be detected even clinically and dermoscopically.

No single clinicodermoscopic feature can allow reliable differentiation of Spitz nevus from melanoma. In patients up to 12 years of age, small (up to 1 cm) and dermoscopically typical spitzoid lesions can be submitted to follow-up with controls every 3 to 6 months; conversely, all spitzoid lesions must be excised in patients older than 12 years.