Melanotic neuroectodermal tumor of infancy in the skull associated with high serum levels of catecholamine

Abstract

The case of a 5-month-old boy with a left retromastoid melanotic neuroectodermal tumor of infancy is presented. The tumor extended from the subcutaneous tissue of the occiput to the cerebellar hemisphere. Histologically, the epidural part of the tumor was composed of undifferentiated neuroblasts, dense connective tissue, and glandular structures lined by melanin-containing cuboidal cells, whereas the subdural part contained differentiated neuroblasts and melanin-containing cells. The preoperative high serum levels of adrenaline, noradrenaline, vanillylmandelic acid, and neuron-specific enolase returned to normal after two operations and two cycles of chemotherapy; however, the dopamine level was mildly elevated. These data and immunohistochemical and ultrastructural findings strongly suggest that melanotic neuroectodermal tumor of infancy is derived from the neural crest.