Melanotic neuroectodermal tumor of infancy (MNTI): A report of two cases from Pakistan

Abstract

Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare tumor of neural crest origin seen in the pediatric population aged less than one year. Despite their very aggressive growth, they are mostly benign in nature, commonly involving the maxilla, but in very rare cases, the skull and brain have also been seen to be affected. We are presenting here two rare cases, including a six months old girl with right orbitotemporal swelling and a three months old boy with right temporal swelling. Both cases were presented consecutively with a gap of one month between April and May 2024 at the Punjab Institute of Neurosciences, Lahore, Pakistan. CT and MRI in both of these cases showed extra-axial lesions. Complete excision of the tumor in both cases was performed with no postoperative complications or recurrence of the tumor at six-month follow-up. The purpose of this case series is to describe these unusual cases of cranial MNTI in infants in order to gain useful insights into the clinical course, diagnostic complexities, and therapeutic techniques for cranial MNTI. This is the first case series on cranial MNTI to be reported from Pakistan.