Sequential ACTH and catecholamine secretion in a phaeochromocytoma

Abstract

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.

Phaeochromocytoma and ACTH‐dependent cushing's syndrome: tumour crf secretion can mimic pituitary cushing's disease

“…Literature review revealed 61 papers and 72 cases of phaeochromocytoma-associated Cushing's syndrome (Table S1). In most cases pathology documented ACTH-positive chromaffin tumour along with adrenocortical hyperplasia, while in three cases pathology confirmed the presence of a mixed cortico- ACTH-secreting [39][40][41][42][43][44] CRF-secreting Small 13 Negative Positive Increased Increased Mondello, et al 20 -Positive Increased -Ruggeri, et al 8 Negative Positive Increased -medullary tumour in which corticosteroid production by the tumour per se appeared a more likely aetiology than ectopic ACTH production [29][30][31] ; in three other cases, [32][33][34] the co-presence of adrenocortical adenoma and a phaeochromocytoma was demonstrated. IPSS was undertaken in only five cases and, all cases, confirmed ectopic ACTH secretion.…”

Ectopic Hormone‐Secreting Pheochromocytoma: A Francophone Observational Study

“…The anti-ACTH antibodies were strongly positive on immunohistochemistry, suggesting that the preoperative test should have been repeated. Symptoms [11]; [12]; [13]; [14]; [15]; [16]; [17]; [18] IL-6 9 [19]; [20]; [21]; [22]; [23]; [24]; [25]; [26]; [27] PTH 2 [28]; [29] Calcitonin 1 [30] VIP 1 [31] GH 1 [32] Calcitonin ? VIP 2 [33]; [34] ACTH ?…”

Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature

“…Abnormal regulation and expression of various genes can be induced by dexamethasone in human-derived pheochromocytoma cells (17). Some tumors, for example, can even change phenotype over time from an ACTHproducing non-catecholamine-secreting tumor to a catecholamine-producing pheochromocytoma (18). Pheochromocytomas that secrete massive amounts of catecholamines on exposure to glucocorticoids may thus represent a specific tumor phenotype expressing altered glucocorticoid receptors and secretory patterns.…”