Despite its kinship with malignant melanoma (melanotic differentiation, ultrastructural evidence of melanosomes, and high frequency of metastases), CCS is genetically different from malignant melanoma or other neuroectodermal or mesenchymal-derived tumors due to the translocation of EWSR1, an uncommon genetic event in melanoma [2,7]. This evidence concerns the gene EWSR1 and melanoma.