Soft tissue

Peripheral nerve

Other benign

Pigmented neuroectodermal tumor of infancy

Topic Completed: 1 November 2012

Minor changes: 20 April 2021

Copyright: 2002-2022,, Inc.

PubMed Search: Pigmented neuroectodermal tumor of infancy

Vijay Shankar, M.D.
Page views in 2021: 1,466
Page views in 2022 to date: 106
Cite this page: Shankar V. Pigmented neuroectodermal tumor of infancy. website. Accessed January 19th, 2022.
Definition / general
  • Also called melanotic neuroectoderrmal tumor of infancy
  • Historically called melanotic progonoma or retinal anlage tumor based on suspected cell of origin
Clinical features
  • Usually benign but may be locally aggressive or undergo malignant change
Case reports
Clinical images

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Anterior region of maxilla

2 month old boy with maxillary tumor

Gross images

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Microscopic (histologic) description
  • Biphasic with tubular or alveolar patterns
  • Large melanin-containing cells with abundant cytoplasm and pale nuclei, surrounding nests of smaller neuroblast-like cells possessing scant or fibrillar cytoplasm
  • Minimal mitotic activity, no atypia
Microscopic (histologic) images

Case #165

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Various stains of mandible lesion

Maxillary tumor

Anterior region of maxilla

6 month old female infant with expansile growth of maxilla

Positive stains
Negative stains
Electron microscopy description
  • Pigmented cells contain melanosomes, neuroblast-like cells contain neurosecretory granules and cytoplasmic processes
Differential diagnosis
  • Neuroblastoma / other small round blue cell tumors or melanoma: typically lack the clinical presentation of a jaw or skull tumor in infants and a biphasic pattern; have prominent necrosis, atypia and mitotic figures
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