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Distinguishing Benign from Malignant Circumscribed Orbital Tumors in Children
Published in Seminars in Ophthalmology, 2018
Yufei Tu, Frederick A. Jakobiec, Katherine Leung, Suzanne K. Freitag
Melanotic neuroectodermal tumor of infancy, also known as retinal anlage tumor or melanotic progonoma, is a rare, osteolytic-pigmented neoplasm of neural crest origin that develops during the first year of life.31 Commonly involving in maxilla, the orbit can rarely be affected. Microscopic examination reveals tubular or alveolar formations of large melanin-containing cells around nests of smaller neuroblastic cells possessing scant or fibrillar cytoplasm.31 Immunohistochemical study usually demonstrates that the tumor cells stain positively for cytokeratin, vimentin, epithelial membrane antigen, S-100, glial fibrillary acidic protein, neuron specific enolase, or HMB-45.32