Sequential ACTH and catecholamine secretion in a phaeochromocytoma.
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.


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.


CONCLUSIONS
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.
van Dam
P Sytze
PS
Department of Clinical Endocrinology, University Medical Center, Utrecht, The Netherlands. P.S.vanDam@digd.azu.nl
van Gils
Ad
A
Canninga-van Dijk
Marijke R
MR
de Koning
Eelco J P
EJ
Hofland
Leo J
LJ
de Herder
Wouter W
WW
D019797:3-Iodobenzylguanidine; D000302:Adrenal Cortex / Q000378:metabolism; D000310:Adrenal Gland Neoplasms / Q000378:metabolism*; D000324:Adrenocorticotropic Hormone / Q000096:biosynthesis / Q000378:metabolism*; D000328:Adult; D002395:Catecholamines / Q000378:metabolism*; D002478:Cells, Cultured; D003366:Cosyntropin / Q000008:administration & dosage; D017077:Culture Media, Conditioned; D006801:Humans; D006854:Hydrocortisone / Q000096:biosynthesis / Q000097:blood; D007205:Indium Radioisotopes; D007457:Iodine Radioisotopes; D008279:Magnetic Resonance Imaging; D008297:Male; D010673:Pheochromocytoma / Q000378:metabolism*; D013004:Somatostatin / Q000031:analogs & derivatives; D014407:Tumor Cells, Cultured
D002395:Catecholamines; D017077:Culture Media, Conditioned; D007205:Indium Radioisotopes; D007457:Iodine Radioisotopes; D003366:Cosyntropin; D019797:3-Iodobenzylguanidine; D013004:Somatostatin; D000324:Adrenocorticotropic Hormone; C081788:pentetreotide; D006854:Hydrocortisone