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Therapeutic Approach

Spitz tumors represent a morphological and biologic spectrum ranging from benign neoplasms to malignant tumors.1,2 On the malignant end of the spectrum, certain spitzoid tumors demonstrate high-grade, pleomorphic features, which make the distinction between these lesions and melanoma challenging.3,4 In between these extremes are a heterogenous group of melanocytic lesions with varying degrees of atypia and unknown malignant potential.3 Rare instances of metastasis and death have been documented, but the malignant potential for these tumors are unquantified and presumed to be low. While evidence-based management guidelines have not been developed, most dermatologists will obtain excision in spitzoid tumors arising in adults and favor a more conservative approach in banal Spitz nevi in children (based on level 4 evidence).

In children, banal Spitz nevi may be diagnosed clinically and confirmed with typical dermoscopy appearance. Spitz nevi may appear deeply pigmented with a characteristic “starburst” dermoscopy pattern, a globular pattern with reticular depigmentation, or with a dotted vessel pattern.5 A conservative approach is often pursued for pediatric patients due to the prevalence of benign tumors and overwhelmingly banal clinical course for Spitz nevi. However, in adults, a typical appearance for a spitzoid proliferation is associated with a surprisingly high risk of melanoma,6 so excision is recommended for adult patients. Figure 108.1 summarizes clinical and dermascopic features of spitzoid tumors, and Figure 108.2 reviews the management of spitzoid tumors.

Figure 108.1

Clinical and dermoscopic features of spitzoid proliferations. (Adapted, with permission, from Bartenstein DW, Fisher JM, Stamoulis C, et al. Clinical features and outcomes of spitzoid proliferations in children and adolescents. Br J Dermatol. 2019;181(2): 366-372.15)

Figure 108.2

Management of suspected spitzoid tumor.

For lesions that are evolving, bleeding, or symptomatic at any age, biopsy is recommended, with intent to remove the base of the lesion because the histopathology at the base of the tumor is vital to determine lesional symmetry, melanocyte maturation, and overall benignity of the lesion. It is not possible to distinguish an atypical Spitz tumor from a spitzoid melanoma clinically. It is exceptionally challenging to distinguish these diagnoses histologically,7 and there are some lesions for which expert dermatopathologist consensus is not achieved. Increasingly, a number of ancillary tests have been explored to characterize spitzoid pigmented lesions.

Fluorescence in situ hybridization (FISH) and comparative genome hybridization (CGH) have been used to detect copy number variant differences that may risk stratify atypical spitz tumors, especially in adults. For instance, homozygous 9p21 deletion—which includes the CDKN2A locus commonly implicated in melanoma pathogenesis—has been associated with aggressive behavior.8 However, FISH has not reliably distinguished between benign Spitz nevi and atypical Spitz tumors in patients younger than 18 ...

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