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The ultrastructure of focal islet cell adenomatosis in the newborn with hypoglycemia and hyperinsulinism

Contribution to the classification of neonatal insulinomas

Summary

In a newborn severe persistent hypoglycemia due to an insulin-producing tumorous proliferation of pancreatic islet cells (insulinoma) was observed. The insulinoma showed the histologic pattern of focal adenomatosis of islet cells. According to the present literature the focal proliferation of islet cell complexes seems to be a frequent and particular feature of insulinomas in the newborn. Differential islet cell staining identified 80%–90% of the proliferated islet cells as B cells. 10%–20% of the cells were found to be A or D cells. Ultrastructurally the majority of the proliferated islet cells were well differentiated B cells. The remaining cells represented either A or D cells or a fourth islet cell type with small spheric granules. Electronmicroscopic evidence of transitions between differentiated islet cells, particularly B cells, and the fourth islet cell type suggests that the fourth islet cell type might represent a precursor cell within the APUD-cell system.

Zusammenfassung

Bei einem Neugeborenen wurde eine schwere persistierende Hypoglykämie als Folge einer Insulin-produzierenden tumorösen Proliferation des Inselzellsystems (Insulinom) beobachtet. Das Insulinom zeigte das histologische Bild einer fokalen Adenomatose der Inselzellen. Nach der vorliegenden Literatur scheint die fokale Proliferation von Inselzellkomplexen ein häufiges und besonderes histologisches Merkmal des neonatalen Insulinoms darzustellen. 80%–90% der proliferierenden Inselzellen konnten mit Hilfe differenzierender Inselzellfärbungen als B-Zellen identifiziert werden, 10%–20% als A- oder D-Zellen. Bei der elektronenmikroskopischen Untersuchung konnte die Mehrzahl der proliferierten Inselzellen als gut differenzierte B-Zellen klassifiziert werden. Die übrigen Zellen repräsentierten entweder A- oder D-Zellen oder einen vierten Inselzelltyp mit kleinen sphärischen Granula. Elektronenmikroskopische Hinweise für Übergangsformen zwischen differenzierten Inselzellen, besonders B-Zellen, und dem vierten Inselzelltyp lassen vermuten, daß der vierte Inselzelltyp eine Vorläuferzelle innerhalb des APUD-Zellsystems darstellt.

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Supported by Deutsche Forschungsgemeinschaft, Sonderforschungsbereich 34, Endocrinology, Hamburg.

The authors are grateful to Prof. Dr. med. J. Kühnau, Department of Internal Medicine, University of Hamburg, Germany, for radioimmunological determination of serum insulin levels. Sincere thanks are also due to Mrs. Katrin Baack and Miss Claudia Drescher for skilful technical assistance.

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Klöppel, G., Altenähr, E. & Menke, B. The ultrastructure of focal islet cell adenomatosis in the newborn with hypoglycemia and hyperinsulinism. Virchows Arch. A Path. Anat. and Histol. 366, 223–236 (1975). https://doi.org/10.1007/BF00427411

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Keywords

  • Hypoglycemia
  • Islet Cell
  • Pancreatic Islet
  • Insulinoma
  • Cell Complex