Melanotic Neuroectodermal Tumor of Infancy Arising in the Temporal Bone

Abstract

Melanotic neuroectodermal tumor of infancy is a rare melanin-containing neoplasm with locally aggressive and rapid expansile growth, usually involving the maxilla, skull, and mandible of early infancy. Radical surgery is critical for a long-term outcome. We present a case of 14-month-old girl with rapid-growing subcutaneous mass arising in the right temporal bone and extending intracranially on computed tomographic scan. Radical surgery was performed. A brownish-black tumor composed of large pigmented epithelioid cells, positive for cytokeratins and HMB-45, and nests of small neuroblast-like cells positive for neuron-specific enolase and synaptophysin, was diagnosed as melanotic neuroectodermal tumor of infancy. The patient remained well without evidence of recurrence for 1 year after surgery. Clinicopathological features, management alternatives and outcome were discussed.

Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature

“…It is essentially a rapid-growing painless tumor causing facial malformation, while the mucosa surface is intact, pigmented and non-ulcerated [ 4 , 6 ]. To the best of our knowledge, there have been few studies focusing on the imaging findings of MNTI to date, and most of the studies were MNTIs from the skull [ 7 – 12 ]. During the past five years, four cases of MNTI occurring in maxillary bone have been confirmed by histopathology in our hospital.…”