Melanotic Neuroectodermal Tumor of Infancy: A Case Report

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign, fast-growing, melanin-containing tumor originating from the neural crest. MNTI can present locally aggressive behavior including bone destruction. A 2-month-old female presented a bluish swelling, with well-defined limits, in the premaxilla. Computed tomography revealed a homogeneous and hypodense image associated with the upper left central primary incisor. The diagnostic hypothesis was dentigerous cyst. An incisional biopsy was performed, and microscopic examination revealed a benign neoplasm exhibiting proliferation of a dual population of cells arranged in solid nests or cords. The first cell type consisted of small rounded cells with scarce cytoplasm, and the second consisted of epithelioid cells containing brown cytoplasmic granules consistent with melanin. Based on these findings, the definitive diagnosis was MNTI. Surgical excision was performed, and there was no tumor recurrence during 1 year of follow-up.