Calvarial melanotic neuroectodermal tumor of infancy: A rare case report

Background: Melanotic neuroectodermal tumor of infancy (MTNI) is a rare congenital tumor of neural crest origin. It is a locally aggressive tumor affecting infants during the first year of life. It commonly arises in the maxilla, mandible, and in the cranial vault. MTNI is usually a benign lesion. Occasionally, it may turn malignant. Though the tumor can be detected by computed tomography and magnetic resonance imaging, an exact diagnosis can only be made by histopathology and immunostaining. Complete surgical excision of the tumor is the gold standard treatment of choice. Benign cranial vault tumors have a good prognosis in contrast to malignant tumors. Case Description: A 3-year-old male child was brought by his mother to the outpatient department with complaints of a large swelling on the left temporal-parieto-occipital region. Computed tomography and magnetic resonance imaging were done which revealed a large hyperdense and hypointense mass in the left temporal-parieto-occipital region with lytic alterations in the skull. The tumor was excised, and tissue sent for histopathology and immunostaining, which revealed a diagnosis of a malignant neuroectodermal tumor of infancy. The patient recovered well in the follow-up period. Conclusion: We report an exceedingly rare case of giant malignant neuroectodermal tumor of infancy (progonoma).