pigmented neuroectodermal tumor of infancy

pigmented neuroectodermal tumor of infancy

A tumor of neural crest origin affecting infants < 6 months of age, seen in the anterior maxilla, oral cavity and skull, less commonly in the mediastinum, thigh, forearm, epididymis Clinical Locally aggressive, 15% recur; few metastasize Lab ↑ VMA in urine. See Pseudonym syndrome.
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