Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report and Review

Abstract

The melanotic neuroectodermal tumor of infancy is a benign but locally aggressive vanillylmandelic acid (VMA) producing neoplasm that originates from neural crest cells. It predominantly affects infants and is most common in the maxilla. Due the anatomical complexity of the head and neck region and the pathological nature of this tumor entity, the surgical therapeutic management poses considerable challenges upon surgeons and patients. The purpose of this article is to report a rare case of a rapidly growing mass, diagnosed as melanotic neuroectodermal tumor of infancy (MNTI), in the right maxilla in a 3-month-old boy which was treated by a radical local resection and followed up after one year without any clinical and radiographical signs of recurrence.