title: Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin.
abstract: We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).
authors.forename: T
authors.initials: T
authors.lastname: Perrone
authors.forename: R K
authors.initials: RK
authors.lastname: Sibley
authors.forename: J
authors.initials: J
authors.lastname: Rosai
mesh_terms: D000328:Adult; D003599:Cytoskeleton / Q000032:analysis; D004379:Duodenal Neoplasms / Q000032:analysis / Q000209:etiology / Q000648:ultrastructure*; D004386:Duodenum / Q000473:pathology*; D004848:Epithelium / Q000473:pathology; D005260:Female; D006801:Humans; D007120:Immunochemistry; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000032:analysis; D010179:Pancreas / Q000473:pathology; D010191:Pancreatic Polypeptide / Q000032:analysis; D010235:Paraganglioma / Q000032:analysis / Q000209:etiology / Q000648:ultrastructure*; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D012583:Schwann Cells / Q000473:pathology; D013194:Staining and Labeling
chemical_list: D009419:Nerve Tissue Proteins; D010191:Pancreatic Polypeptide; D010751:Phosphopyruvate Hydratase