Melanotic neuroectodermal tumor of infancy

Melanotic neuroectodermal tumor of infancy is a rare neoplasm of neural cre st origin. lt almost always occurs in children younger than one year of age with a predilection for the anterior maxillary ridge. Although it usually follows a benign course, local destruction as well as metastasis and death may result from the tumor. Melanotic neuroectodermal tumor is most commonly treated by surgery. 10-15% of the lesions recur. We report the case of a 2 -month-old infant suffering from melanotic neuroectodermal tumor of infancy of the premaxilla who was successfully treated with a combination of vario us chemotherapeutic agents in order to spare the child a mutilating operati on or the late effects of radiation. Discussion: Chemotherapeutic management of melanotic neuroectodermal tumor of infancy can possibly spare the child a mutilating operation or at least reduce its extent.