Ear Nose Throat J. 2006 Jun;85(6):365.
FIRST PARAGRAPH: Melanotic neuroectodermal tumor of infancy is a rare, neural-crest-derived neoplasm that is believed to be congenital. The tumor has a marked predilection for the head and neck — particularly the maxilla, where approximately 70% of these tumors are located. The anterior maxilla is most commonly affected. There is no predilection for either sex. Nearly all patients present with an enlarging mass, usually within a few years of birth. Intraoral lesions may appear ‘blue,’ suggesting the presence of pigment. Radiographic images will often show a destructive lesion with tooth displacement, but they are nonspecific. Laboratory studies show high urinary levels of vanillylmandelic acid.
PubMed ID: 16866106
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