Melanotic neuroectodermal tumor of infancy-A neoplasm of neural crest origin. Report of a case associated with high urinary excretion of vanilmandelic acid

A case of melanotic neuroectodermal tumor of infancy (melanotic progonoma, retinal anlage tumor, melanoameloblastoma) associated with high urinary excretion of vanilmandelic acid is presented for the first time and discussed in the light of probable origin from the neural crest. The VMA levels returned to normal after removal of the tumor. With regard to pathogenesis and its very frequent occurrence in the upper anterior jaw, the authors hypothesize that the tumor is not, in fact, odontogenic in origin as has been suggested but may arise from neural crest cells which migrate in many cases with the odontoblasts that form the dentin of the maxillary anterior teeth.