Paraganglioma
Mari Mino-Kenudson, MD
Key Facts
Terminology
Gangliocytic paraganglioma
Uncommon (approximately 1% of ampullary neoplasms)
Some cases associated with NF-1
Clinical Issues
Most commonly involve periampullary duodenum
Clinically benign
Microscopic Pathology
Epithelioid, spindled, and ganglion-like cells present in varying proportions
Endocrine cells may also be present
S100 may reveal sustentacular-like cells surrounding nests of epithelioid cells
No mitoses or necrosis
 Periampullary gangliocytic paragangliomas often have an infiltrating pattern at the periphery. The lesion is centered in the submucosa with extension into the overlying mucosa. |
 Gangliocytic paragangliomas consisting of a mixture of epithelioid cells, ganglion-like cells  , and nerve sheath elements  . |
TERMINOLOGY
Synonyms
Gangliocytic paraganglioma
Nonchromaffin paraganglioma
Definitions
Neoplasm consisting of epithelioid, ganglion-like cells, and nerve sheath elements
Associated with neurofibromatosis type 1 (NF1) in some cases
ETIOLOGY/PATHOGENESIS
Origin
Several theories
Progenitor neural crest cells
Embryonic celiac ganglion
Endodermally derived epithelial cells originating from the ventral primordium of pancreas
(hamartomatous proliferation)
Pancreatic tumor composed of ganglion-islet cell complexes
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