An unusual occurrence of melanotic neuroectodermal tumor of infancy with malignant features

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, commonly benign, congenital pigmented neoplasm that originates from the neural crest. In contrast to peripheral MNTI usually found in the maxilla, cerebral MNTI has a much worse outcome. We report a case of intracranial MNTI with malignant features in a 2-year-old child. The clinical, radiological, histological, and immunohistochemical findings are described, and the published data on this rare entity are reviewed.