Progenitor neural crest cells
Embryonic celiac ganglion
Endodermally derived epithelial cells originating from ventral primordium of pancreas (hamartomatous proliferation)
Pancreatic tumor composed of ganglion-islet cell complexes
Clinical Issues
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3rd-9th decades (mean age: 6th decade)
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Men slightly out number women
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Most commonly involve periampullary duodenum
Microscopic
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Epithelioid, spindled, and ganglion-like cells present in varying proportions
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Endocrine cells may also be present
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S100 may reveal sustentacular-like cells surrounding nests of epithelioid cells
TERMINOLOGY
Synonyms
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Gangliocytic paraganglioma
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Nonchromaffin paraganglioma