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Melanotic Neuroectodermal Tumor of Infancy

Background

Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants.
The lesion has had an interesting history since its initial description by Krompecher in 1918 as a congenital melanocarcinoma.

For the next 5 decades, the lesion was reported under a variety of different names as succeeding authors attempted to identify the cell of origin. Some of the terms applied to this lesion included pigmented ameloblastoma, retinal anlage tumor, melanotic progonoma, melanotic adamantinoma, and pigmented epulis of infancy.
These terms reflected theories of suspected origin from the odontogenic apparatus, the pigmented anlage of the retina, or the sensory neuroectodermal tissues.

In 1966, Borello and Gorlin reported a case with high urinary excretion of vanillylmandelic acid (VMA), suggesting a neural crest origin, and they proposed the term melanotic neuroectodermal tumor of infancy.
Since then, numerous histochemical, immunohistochemical, electron microscopic, and tissue culture studies have supported the neural crest origin and confirmed the preferred term of melanotic neuroectodermal tumor of infancy.

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