Immunohistochemical, ultrastructural, and histogenetic considerations in a patient with melanotic neuroectodermal tumor of infancy

Abstract

The melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon neoplasm of early childhood that has been referred to by a variety of names in the past, reflecting conflicting opinions about its histogenesis. 1–6 Typically, the tumor arises in the jaws, especially in the maxilla of infants under 12 months old. It usually behaves in a benign fashion, but a few cases of recurrence and metastasis have been described in the literature. 7–9 Krompecher, 1 in 1918, published the first report of the tumor, and it was only in 1966 that Borello and Gorlin 6 reported that the tumor elaborated high levels of vanilmandelic acid, suggesting a neural crest origin because this laboratory finding is shared with other tumors originating from the neural crest: pheochromocytoma, ganglioneuroblastoma, neuroblastoma, and retinoblastoma. This fact led the authors to propose the term melanotic neuroectodermal tumor of infancy for the lesion. In recent years, a few ultrastructural and immunohistochemical studies have been performed to clarify the histogenesis of the tumor. Nevertheless, the results are still inconclusive. 10–15 For this reason, in the present study we examined a case of the tumor using immunohistochemical and ultrastructural observations.