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Cushing’s syndrome with intermittent ectopic ACTH production

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Abstract

A case of ectopic ACTH syndrome with intermittent secretion in a 72-yr-old woman is described. Plasma and urinary Cortisol levels were obtained at frequent intervals for a period of more than 10 months and varied erratically from the normal range to extremely high values. Nonsuppression by high doses of dexamethasone was documented during a period of hypersecretion. Normal circadian rhythmicity and normal responses to hypoglycemia were observed during an interval of dormance of the ectopic secretion. Hypokalemia did not develop. These findings, together with the occult nature of the primary tumor, resulted in unusual diagnostic difficulties. Liver masses were detected by echography and CT scan. Pathological examination of liver biopsies suggested a neuroendocrine tumor of foregut origin. While a multicentric primary apudoma secreting ACTH was a putative diagnosis, detailed and extensive microscopic post-mortem studies revealed a more likely primary tumor site in the pancreatic tail.

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Author information

Author notes

  1. E. Laurent M.D. (Research Fellow) & O. van Reeth (Research Fellows)

    Present address: National Fund for Scientific Research, Belgium

Authors and Affiliations

  1. Department of Endocrinology, Free University of Brussels, Belgium

    A. van Coevorden & J. Mockel

  2. Department of Pathology, Free University of Brussels, Belgium

    F. Rickaert

  3. Institute of Interdisciplinary Research, Campus Erasme, Bât C., Free University of Brussels, Route de Lennik 808, B-1070, Brussels, Belgium

    E. Laurent M.D. (Research Fellow)

  4. Laboratory of Neuropathology and Neuropeptides Research, Campus Erasme, Free University of Brussels, Belgium

    O. van Reeth (Research Fellows)

  5. Department of Medicine, The University of Chicago, Illinois, USA

    E. Van Cauter

Authors
  1. A. van Coevorden
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  2. E. Laurent M.D.
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  3. F. Rickaert
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  4. O. van Reeth
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  5. E. Van Cauter
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Additional information

Supported in part by the Belgian F. R. S. M. (86–89; 34523) and F.M.R.E. (86–89), by the Fondation Reine Elisabeth, by the APMO Foundation and by a grant “Actions Concertees” from the Belgian Ministry of Scientific Policy.

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van Coevorden, A., Laurent, E., Rickaert, F. et al. Cushing’s syndrome with intermittent ectopic ACTH production. J Endocrinol Invest 13, 317–326 (1990). https://doi.org/10.1007/BF03349570

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  • DOI: https://doi.org/10.1007/BF03349570

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