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Pancreatic glucagonoma with and without syndrome

Immunocytochemical study of 5 tumour cases and review of the literature

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Summary

In five patients single or multiple glucagonomas were characterized by immunocytochemistry. Two large single glucagonomas were associated with the glucagonoma syndrome, which completely dissappeared after removal of the tumours. The morphologic findings in these patients are compared with 48 others collected from literature.

In the other three patients, the glucagonomas were not associated with a clinical syndrome and were detected by chance (one accompanying an insulinoma; the other in pancreases of patients suffering from multiple endocrine neoplasia I; MEN I). These tumours appeared by their histological, immunocytochemical and ultrastructural features better organized than the glucagonomas with syndrome.

Glucagonomas not producing a syndrome can be classified into (a) solitary, often malignant endocrine pancreatic tumours, (b) glucagonomas associated with insulinomas and other tumours, (c) multiple glucagonomas in MEN I and (d) single microglucagonomas in elderly patients. It is emphasized that only immunohistology allows clear identification of these tumours as glucagonomas.

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Authors and Affiliations

  1. Institute of Pathology, University of Hamburg, Martinistr. 52, D-2000, Hamburg 20, Germany

    E. Ruttman, G. Klöppel, G. Bommer, M. Kiehn & Ph. U. Heitz

  2. Institutes of Pathology, Universities of Basel, Switzerland

    E. Ruttman, G. Klöppel, G. Bommer, M. Kiehn & Ph. U. Heitz

  3. Department of Dermatology, University of Hamburg, Germany

    E. Ruttman, G. Klöppel, G. Bommer, M. Kiehn & Ph. U. Heitz

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  1. E. Ruttman
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  2. G. Klöppel
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  4. M. Kiehn
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  5. Ph. U. Heitz
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Ruttman, E., Klöppel, G., Bommer, G. et al. Pancreatic glucagonoma with and without syndrome. Virchows Arch. A Path. Anat. and Histol. 388, 51–67 (1980). https://doi.org/10.1007/BF00430676

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