Melanotic neuroectodermal tumor of infancy

a benign neoplasm of neuroectodermal origin that most often involves the anterior maxilla of infants in the first year of life. It presents clinically as a rapidly growing blue-black lesion producing a destructive radiolucency; histologically, it is characterized by small, round, undifferentiated tumor cells interspersed with larger polyhedral melanin-producing cells arranged in an alveolar configuration. SYN: melanoameloblastoma, pigmented ameloblastoma, pigmented epulis, progonoma of jaw, retinal anlage tumor.