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progonoma of jaw --> melanotic neuroectodermal tumour 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 characterised by small round undifferentiated tumour cells interspersed with larger polyhedral melanin-producing cells arranged in an alveolar configuration.

Synonym: melanoameloblastoma, pigmented ameloblastoma, pigmented epulis, progonoma of jaw, retinal anlage tumour.