Nesidioblastosis
Vikram Deshpande, MD
Key Facts
Terminology
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Morphologic findings associated with functional disorders of β cells (hyperinsulinemic hypoglycemia) in absence of insulinoma
Etiology/Pathogenesis
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Abnormalities in potassium channels
Clinical Issues
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Persistent neonatal hypoglycemia
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Adult NB very rare
Microscopic Pathology
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Neonatal focal NB
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Confluent or partially confluent clusters of endocrine cells
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Neonatal diffuse NB
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Giant endocrine cells (3x larger than normal endocrine cells)
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This image shows predominantly unremarkable pancreatic acinar tissue. Large and back to back islet celllike structures  are present, a finding that suggests focal nesidioblastosis. |
Focal nesidioblastosis features confluent growth of endocrine cells  . The endocrine cells are very similar to those seen in normal pancreatic islets. Note the intervening normal acinar cells  . |
TERMINOLOGY
Abbreviations
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Nesidioblastosis (NB)
Definitions
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Morphologic findings associated with functional disorders of β cells (hyperinsulinemic hypoglycemia) in absence of insulinoma
ETIOLOGY/PATHOGENESIS
Potassium Channel Abnormalities
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ACCC8 (formerly SUR1)
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KCNJ11 (formerly Kir6.2)
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