Eur J Endocrinol
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DOI: 10.1530/eje.0.1470201
European Journal of Endocrinology, Vol 147, Issue 2, 201-206
Copyright © 2002 by European Society of Endocrinology
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Case Reports

Sequential ACTH and catecholamine secretion in a phaeochromocytoma

PS van Dam, A van Gils, MR Canninga-van Dijk, EJ de Koning, LJ Hofland, and WW de Herder

Department of Clinical Endocrinology, University Medical Center, Utrecht, The Netherlands. P.S.vanDam@digd.azu.nl

OBJECTIVE: We describe a patient with an ACTH-producing phaeochromocytoma who initially presented with hypercortisolism and normal catecholamine concentrations, followed by near-normalisation of ACTH secretion and massive catecholamine secretion. In vitro studies were carried out on the tumour to evaluate the interaction between the tumour cells and normal adrenal cortex. METHODS AND RESULTS: A 30-year-old man initially presented with severe hypercortisolism, biochemical evidence of ectopic ACTH production, a tumour in the right adrenal gland without a hyperintense signal on the T2-weighted images at magnetic resonance imaging (MRI) scanning, and normal urinary metanephrine concentrations. After 6 months, ACTH production had almost completely resolved, but the patient developed severe hypertension and excess catecholamines. At repeated MRI-scanning, the T2-weighted images showed a hyperintense signal, in agreement with the diagnosis of phaeochromocytoma. Although the initial T1-weighted images suggested bleeding in the adrenal tumour, no signs of bleeding were observed after surgical removal. The diagnosis of ACTH-producing phaeochromocytoma was histologically and immunohistochemically confirmed. Cultured cell suspensions of the tumour secreted ACTH, which stimulated cortisol production in the ipsilateral adrenocortical cells. CONCLUSION: This case demonstrates that the biological activity of an ACTH-producing phaeochromocytoma can vary significantly in time, which may be the consequence of different stages of tumour differentiation.


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A. L Rosas, A. A Kasperlik-Zaluska, L. Papierska, B. L. Bass, K. Pacak, and G. Eisenhofer
Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature
Eur. J. Endocrinol., March 1, 2008; 158(3): 423 - 429.
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