Soft tissue
Peripheral nerve
Pigmented neuroectodermal tumor of infancy

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 21 September 2016, last major update November 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Pigmented neuroectodermal tumor of infancy

Definition / General
Terminology
  • 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
Treatment
Clinical Images

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Maxilla

Anterior region of maxilla

2 month old boy with maxillary tumor

Gross Images

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Maxilla

Micro 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
Micro Images

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Case of Week #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