Pancreas
Congenital anomalies
Nesidioblastosis


Topic Completed: 1 August 2012

Minor changes: 22 October 2020

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PubMed Search: Nesidioblastosis[TI] pancreas[TIAB]

Deepali Jain, M.D.
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Cite this page: Jain D. Nesidioblastosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasnesidio.html. Accessed October 26th, 2020.
Definition / general
  • Islets in intimate association with ducts, with formation of ductuloinsular complexes
  • Also called congenital islet hyperplasia
  • Indicates active formation of endocrine cells by multipotential cells in basal layer of ducts
Clinical features
  • Normal in infants, exaggerated in neonatal hyperglycemia (infants of diabetic mothers)
  • In adults, is rare cause of persistent hyperinsulinemic hypoglycemia
  • Associated with Beckwith-Weidemann syndrome, chronic pancreatitis, cystic fibrosis, endocrine neoplasms, gastric bypass patients (Mod Pathol 2009;22:239), Zollinger-Ellison syndrome
  • May be due to increase in expression of growth factors IGF2, IGF1Ra and TGFBR3 in islets (Mod Pathol 2009;22:239)
  • Must rule out mutation in HADH gene (J Clin Endocrinol Metab 2011;96:E498)
  • Either focal or diffuse (Am J Surg Pathol 1989;13:766):
    • Focal: nodular hyperplasia of islet-like cell clusters, including ductuloinsular complexes and hypertrophied insulin cells with giant nuclei
    • Diffuse: involves entire pancreas; irregularly sized islets and ductuloinsular complexes present, both contain hypertrophied insulin cells, peliosis type vascular ectasia
Case reports
Treatment
  • Focal nesidioblastosis: partial pancreatectomy with excision of diseased areas; diffuse: near total pancreatectomy
Microscopic (histologic) description
  • Islets in intimate association with ducts, with formation of ductuloinsular complexes
  • Beta cell hypertrophy (Am J Surg Pathol 2005;29:524)
Microscopic (histologic) images

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20 day old girl with persistent hypoglycemia and seizures


23 year old man with hypoglycemia mimicking an insulinoma

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