Melanotic neuro-ectodermal tumour of infancy. A case report

A case of melanotic neuro-ectodermal tumour of infancy (retinal anlage tumour, melanotic ameloblastoma) is described. Although a well recognized entity, the biological origin of this tumour is still disputed. A confusing array of names has been awarded to this tumour which characteristically occurs in infants under one year of age, commonly arising from the maxilla but also from the mandible, frontal bone, mediastinum, shoulder and cerebellum. While the origin of the tumour remains unclear, the ultra-structural evidence of Haywood et al. (1969) supports a neural crest origin. Consistent with this are the findings by Borello and Gorlin (1966) of high urinary vanillylmandelic acid excretion in one case and the correlation of tumour enzyme patterns with those of paragangliomas, neuroblastomas and phaeochromocytomas.