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.

• Authors: Jian-guo Zhang, Rui-jiao Zhao, Ling-fei Kong
• Journal: Journal of child neurology (J Child Neurol) Vol. 30 Issue 5 Pg. 631-4 (Apr 2015) ISSN: 1708-8283 [Electronic] United States
• PMID: 23965399 (Publication Type: Case Reports, Journal Article)
• Copyright: © The Author(s) 2013.

Topics

• Diagnosis, Differential
• Female
• Follow-Up Studies
• Humans
• Infant
• Neuroectodermal Tumor, Melanotic (diagnosis, diagnostic imaging, pathology, surgery)
• Skull Neoplasms (diagnosis, diagnostic imaging, pathology, surgery)
• Temporal Bone (diagnostic imaging, pathology, surgery)
• Tomography, X-Ray Computed
• Treatment Outcome