The melanotic neuroectodermal tumor of infancy. Report of two cases and review of the literature.

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with an alarming but classical clinical presentation. It appears as a rapidly enlarging mass in the jaws of skull of infants and unless MNTI is considered in the differential diagnosis, the lesion can easily be mistaken for a malignant neoplasm. Although possessing an aggressive growth rate and radiographic appearance, the MNTI almost always behaves in a benign fashion and can be treated with local excision. However, recent reports of malignant behavior as well as of occasional recurrences make close follow-up important. Approximately 139 cases of the MNTI have been reviewed and tabulated with respect to age at discovery, sex, tumor location, length of follow-up, and whether recurrence occurred, 2 additional cases, 1 of which was in the mandible, are presented along with theories of origin and recommended therapy.