Pigmented Epithelioid Melanocytoma in a Child: Unusual Clinical Presentation

Zembowicz et al[1] coined the term PEM for a “low-grade melanocytic tumor with metastatic potential indistinguishable from animal-type melanoma and epithelioid blue nevus”. PEM is a distinct melanocytic tumor occurring in a sporadic setting and in the context of Carney complex. A 10 year-old child was referred for evaluation of a blue-black cutaneous macule found in the right pectoral region. The lesion measured 0.4×0.3 cm. The histology of the lesion showed a densely pigmented dermal nodule with infiltrative borders. The proliferation was composed of epithelioid and spindle melanocytes with heavy pigmentation; atypical cells were present (Fig. 1).