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Multihormonal, amyloid-producing tumour of the islets of Langerhans in a twelve year old boy

Clinical, morphological and biochemical data and review of the literature

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Summary

Clinical, pathological and biochemical data from a twelve year old boy with hypoglycaemic attacks and hyperinsulinism are presented. A pancreatic islet cell adenoma was diagnosed by ultrasonic scanning and enucleated from the head of the pancreas. Histological study revealed a mixed cellular population consisting of A1- and B-cells. Large amounts of extravascular amyloid deposited were found between the cell strands. The tumour extract produced a prompt elevation of gastric acid secretion in the rat gastrin bio-assay. It is concluded that this islet cell tumour was a combined gastrinoma-insulinoma. The possible pathogenesis of this variety of the multiple endocrine adenoma syndrome is discussed. Monocellular-multihormonal islet cell tumours might arise from biochemically multipotent endocrine stem cells, whereas the multicellular-multihormonal type could either be a regulatory neoplasia or be derived from different neuroendocrine cells.

Zusammenfassung

Bei einem übergewichtigen 12jährigen Knaben mit Hypoglykämieanfällen und erhöhtem Blutinsulinspiegel konnte durch Ultraschall ein Tumor des Pankreaskopfes dargestellt werden. Das exstirpierte Gewebe erwies sich histologisch als ein aus A1- und B-Zellen aufgebautes Inselzelladenom mit ausgedehnten extravasculären Amyloidablagerungen. Ein Extrakt aus dem Tumorgewebe bewirkte eine sofortige Zunahme der Magensäuresekretion am perfundierten Rattenmagen. Diagnose: Insulinom-Gastrinom. Monocellulärplurihormonelle Inselzelltumoren leiten sich möglicherweise von biochemisch pluripotenten Stammzellen der Langerhansschen Inseln her. Pluricellulär-plurihormonelle Adenome sind hingegen eher als Regulationsgeschwülste aufzufassen, könnten aber auch von verschiedenen neuroendokrinen Zellen abstammen.

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  1. H. Steiner

    Present address: dept. of experimental endocrinology, Istituto di Farmacologia e della Terapia, Via Vanvitelli 32, I-20129, Milano

Authors and Affiliations

  1. Department of Pathology, University of Basel, Switzerland

    Ph. Heitz, H. Steiner, F. Halter, F. Egli & J. P. Kapp

  2. the Department of Pathology, University of Lausanne, Switzerland

    Ph. Heitz, H. Steiner, F. Halter, F. Egli & J. P. Kapp

  3. the Department of Medicine, University Hospital of Bern, Switzerland

    Ph. Heitz, H. Steiner, F. Halter, F. Egli & J. P. Kapp

  4. the Department of Pediatrics, University Hospital of Basel, Switzerland

    Ph. Heitz, H. Steiner, F. Halter, F. Egli & J. P. Kapp

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Heitz, P., Steiner, H., Halter, F. et al. Multihormonal, amyloid-producing tumour of the islets of Langerhans in a twelve year old boy. Virchows Arch. Abt. A Path. Anat. 353, 312–324 (1971). https://doi.org/10.1007/BF00549044

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