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The Pluriglandular Syndromes

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Abstract

The rare hereditary syndromes of endocrine adenomatosis have been known to pathologists since the turn of the century (Erdheim, 1903). Clinicians have been able to diagnose the syndrome for the past 30 years (Rossier, 1939; Shelbourne, 1945; Underdahl, 1953; Wermer, 1954), and about 100 cases have now been described (Ballard, 1964; Johnson, 1967). There is a hypothesis concerning the pathogenesis of these syndromes which also takes into account the occasional occurence of ectopic peptide hormones in certain types of endocrine adenomatosis (Weichert, 1970). It is proposed that neuroendocrine cells migrate to the primitive gastrointestinal tract from where they form the endocrine cells of the anterior pituitary and the parathyroid glands and islet cells of the pancreas. Neuroendocrine cells, as argentaffin cells, are scattered throughout the gastrointestinal tract mucosa and also occur in other organs such as the bronchi.

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Labhart, A. (1974). The Pluriglandular Syndromes. In: Clinical Endocrinology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-96158-8_18

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