INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNT1) is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, and rapidly growing that develops during the first year of life. It most commonly arises from the maxilla, the cranial vault, and the mandible. Early diagnosis and radical surgery are critical for a long-term outcome.
METHODS: A literature search through PUBMED revealed 43 cases of MNT1 arising in the skull. We reviewed the available literature and studied the presenting symptoms, diagnostic procedures, treatment, rates of recurrences, malignancy, and data of follow-up. We report two further cases of infants aged 4 and 10 months, respectively, with MNT1 arising from the cranial vault who underwent radical excision of the lesion.
CONCLUSION: Melanotic neuroectodermal tumor of infancy should be included in the differential diagnosis of skull lesions in infants. Radical surgery must be considered as the treatment of choice and close follow-up for at least 2 years is necessary.