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. |
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 complexesStay updated, free articles. Join our Telegram channel
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