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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Pigmented neuroectodermal tumor of infancy
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 13 February 2010, last major update – February 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Uncommon, rapidly growing mesenchymal tumor of jaw or skull in infants, with biphasic histology of melanin containing cells and neuroblast-like cells (eMedicine)
Terminology
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● Historically called melanotic progonoma or retinal anlage tumor based on suspected cell of origin
Epidemiology
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● Uncommon tumor of infants
Etiology
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● In 1966, Borello and Gorelin reported a patient with high urinary excretion of vanilmandelic acid, suggesting a neuroectodermal origin (Cancer 1966;19:196), which has subsequently been confirmed
Clinical
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● Rapidly growing mesenchymal tumor that typically occurs in the bones of the jaw or skull in infants.
Case reports
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● 4 month old child with midline skull lesion (Case of the Week #165)
Treatment
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● Excision with negative margins
● Recur in 37% of cases, metastasis in 7%, although no prognostic factors have been identified (Fetal Pediatr Pathol 2006;25:59)
Micro description
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● 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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Low power High power
Positive stains
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● Larger cells: keratin and HMB45
● Smaller cells: usually CD57/Leu7 and NSE
Negative stains
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● Both cell types are usually negative for S100 (Am J Surg Pathol 1993;17:566)
Electron microscopy
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● Pigmented cells contain melanosomes, neuroblast-like cells contain neurosecretory granules and cytoplasmic processes
Differential diagnosis
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● 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
End of Soft Tissue Tumors Part 3 > Peripheral nerve tumors > Pigmented neuroectodermal tumor of infancy
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