Heterotopic pancreas, periampullary somatostatinoma and type I neurofibromatosis: a pathogenetic proposal.

Abstract

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.

• Authors: R Chetty, A Essa
• Journal: Pathology (Pathology) Vol. 31 Issue 2 Pg. 95-7 (May 1999) ISSN: 0031-3025 [Print] England
• PMID: 10399162 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Ampulla of Vater (pathology)
• Choristoma (complications, pathology)
• Duodenal Neoplasms (complications, pathology)
• Fatal Outcome
• Humans
• Male
• Neoplasms, Multiple Primary (complications, pathology)
• Neurofibromatosis 1 (complications, pathology)
• Pancreas
• Somatostatinoma (complications, pathology)