A Rare Case of Well-Differentiated Neuroendocrine Tumor Arising From a Jejunal Pancreatic Heterotopia

Ryan L. Sappenfield, Kathleen Byrnes

Research output: Contribution to journalArticlepeer-review


Pancreatic heterotopia is a well-described entity occurring at multiple abdominal sites, most commonly the stomach and small intestine. They can develop similar disease processes as the pancreas ranging from acute pancreatitis, cyst formation, or neoplasms, most commonly ductal adenocarcinoma. Neuroendocrine tumors (NETs) arising in pancreatic heterotopias are exceedingly rare with only 3 prior published cases. In this article, we describe the first reported case of a NET arising in a jejunal pancreatic heterotopia in a 59-year-old woman presenting with abdominal pain and diarrhea. The submucosal tumor was composed of a well-differentiated NET (World Health Organization grade 1) directly intermixed with a heterotopic pancreas consisting of acinar cells, islet cells, and ducts. This case illustrates that NETs can occur in association with pancreatic heterotopias at any site. Also, the importance for pathologists to recognize that pancreatic heterotopias can give rise to a variety of neoplasms and is not limited to ductal adenocarcinoma.

Original languageEnglish (US)
Pages (from-to)190-193
Number of pages4
JournalInternational journal of surgical pathology
Issue number2
StatePublished - Apr 2021
Externally publishedYes


  • case report
  • heterotopic pancreas
  • jejunum
  • neuroendocrine tumor
  • small intestine

ASJC Scopus subject areas

  • Surgery
  • Anatomy
  • Pathology and Forensic Medicine

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