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Pancreas

Congenital anomalies

Nesidioblastosis

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 22 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● 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
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● Normal in infants, exaggerated in neonatal hyperglycemia (infants of diabetic mothers)
● In adults, is rare cause of persistent hyperinsulinemic hypoglycemia
● Associated with Beckwith-Weideman 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
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● 44 year old man with symptomatic hypoglycemia and localized nesidioblastosis (Hum Pathol 2010;41:447)

Treatment
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● Focal nesidioblastosis: partial pancreatectomy with excision of diseased areas; diffuse: near total pancreatectomy

Micro description
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● Islets in intimate association with ducts, with formation of ductuloinsular complexes
● Beta cell hypertrophy (Am J Surg Pathol 2005;29:524)

Micro images
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Various images

   
20 day old girl with persistent hypoglycemia and seizures

     
23 year old man with hypoglycemia mimicking an insulinoma

 
Left-expansion of endocrine cells from a ductal structure forming a ductulo-insular complex (arrow)
Right-irregular hyperplastic islets (arrows) in close approximation with pancreatic exocrine ductal structures

 
Left-chromogranin staining within irregular islets
Right-insulin staining within islets

End of Pancreas > Congenital anomalies > Nesidioblastosis

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