Heterotopic pancreas, periampullary somatostatinoma and type I neurofibromatosis: a pathogenetic proposal.
This case documents the association of ectopic pancreatic tissue with a duodenal somatostatinoma in a patient with type I neurofibromatosis. Pancreatic ducts have been noted within the centres of somatostatinomas, but little significance has been attached to this finding. Here we describe a patient in whom a separate proliferation of somatostatin cells occurred in association with the ectopic pancreatic ductular epithelium. This lesion bore a striking resemblance to the ductulo-insular or ductulo-endocrine complexes that are seen in nesidioblastosis in the pancreas. We therefore postulate that the ducts, which are sequestered within somatostatinomas, are of pathogenetic significance. The somatostatin-producing cells arise from these ducts, very much in the fashion of ductulo-endocrine complexes in nesidioblastosis.
Department of Pathology, University of Natal School of Medicine, King Edward VIII Hospital, Durban, South Africa.
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D000328:Adult; D014670:Ampulla of Vater / Q000473:pathology*; D002828:Choristoma / Q000150:complications / Q000473:pathology*; D004379:Duodenal Neoplasms / Q000150:complications / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications / Q000473:pathology*; D010179:Pancreas; D013005:Somatostatinoma / Q000150:complications / Q000473:pathology*