Focal hyperinsulinism in ectopic pancreatic tissue: preoperative localization and curative resection
5 affiliations
- 1Division of Pediatric Endocrinology and Diabetes, University of Alabama at Birmingham, Birmingham, AL 35233, USA.
- 2Texas College of Osteopathic Medicine, UNT Health Fort Worth, Fort Worth, TX 76107, USA.
- 3Division of Pediatric Endocrinology and Diabetes, Cook Children's Medical Center, Fort Worth, TX 76104, USA.
- 4Division of Pathology, Cook Children's Medical Center, Fort Worth, TX 76104, USA.
- 5Clinical and Translational Science, Cook Children's Health Care System, Fort Worth, TX 76104, USA.
Abstract
Ectopic pancreas (EP) is pancreatic tissue located outside the native pancreas. Most cases are asymptomatic and identified incidentally. Only a few cases of hyperinsulinism (HI) arising from EP have been reported. We describe an infant who had neonatal hypoglycemia which was not shown to be resolved and later presented at 5 months of age with recurrent abnormal limb movements associated with severe hypoglycemia. Following failure of diazoxide therapy, genetic testing identified a paternally inherited pathogenic variant in ABCC8. Preoperative fluorine-18-L-dihydroxyphenylalanine positron emission tomography/computed tomography (¹⁸F-DOPA PET/CT) revealed a focal area of uptake in the distal duodenum/proximal jejunum consistent with ectopic pancreatic tissue. Surgical resection of this lesion resulted in complete resolution of hypoglycemia while preserving the native pancreas. We review previously reported cases of congenital HI arising in ectopic pancreatic tissue, highlighting the novelty of preoperative ¹⁸F-DOPA PET/CT localization that enabled curative resection during the initial surgery for our patient.
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BibTeX
@article{resnick2026focal,
title = {{Focal hyperinsulinism in ectopic pancreatic tissue: preoperative localization and curative resection}},
author = {Ortal Resnick and Ashley Mathew and Lisa Truong and Stacey Berry and Deborah Rafferty and Paul S Thornton},
journal = {JCEM case reports},
year = {2026},
month = oct,
doi = {10.1210/jcemcr/luag338},
url = {https://europepmc.org/article/PMC/PMC13717193},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Focal hyperinsulinism in ectopic pancreatic tissue: preoperative localization and curative resection
AU - Ortal Resnick
AU - Ashley Mathew
AU - Lisa Truong
AU - Stacey Berry
AU - Deborah Rafferty
AU - Paul S Thornton
PY - 2026
DA - 2026/10/29
JO - JCEM case reports
DO - 10.1210/jcemcr/luag338
UR - https://europepmc.org/article/PMC/PMC13717193
AB - Ectopic pancreas (EP) is pancreatic tissue located outside the native pancreas. Most cases are asymptomatic and identified incidentally. Only a few cases of hyperinsulinism (HI) arising from EP have been reported. We describe an infant who had neonatal hypoglycemia which was not shown to be resolved and later presented at 5 months of age with recurrent abnormal limb movements associated with severe hypoglycemia. Following failure of diazoxide therapy, genetic testing identified a paternally inherited pathogenic variant in ABCC8. Preoperative fluorine-18-L-dihydroxyphenylalanine positron emission tomography/computed tomography (¹⁸F-DOPA PET/CT) revealed a focal area of uptake in the distal duodenum/proximal jejunum consistent with ectopic pancreatic tissue. Surgical resection of this lesion resulted in complete resolution of hypoglycemia while preserving the native pancreas. We review previously reported cases of congenital HI arising in ectopic pancreatic tissue, highlighting the novelty of preoperative ¹⁸F-DOPA PET/CT localization that enabled curative resection during the initial surgery for our patient.
SN - 2755-1520
ER -