Histopathology of the Pancreas in Congenital Hyperinsulinism

  • Tricia R. BhattiEmail author
  • Eduardo D. Ruchelli
Part of the Contemporary Endocrinology book series (COE)


The underlying genetic alterations in congenital hyperinsulinism (HI) are reflected in characteristic histopathologic changes in the pancreas of affected patients. The majority of cases with inactivating KATP mutations can be segregated into two morphologic forms, diffuse and focal HI, with corresponding alterations in islet cell nuclear size and growth patterns, respectively. Hyperinsulinism due to other gene defects and syndromes is less common, but these patients also demonstrate abnormalities in pancreatic endocrine tissue morphology. Correlation of the histologic and genetic findings remains a mainstay of both diagnosis and patient management and has especially important clinical implications at the time of surgery.


Focal HI Diffuse HI Nucleomegaly Overgrowth syndrome p57 immunohistochemistry 


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineThe Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaUSA

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