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Endocrine Abstracts (2015) 37 GP02.08 | DOI: 10.1530/endoabs.37.GP.02.08

Endocrine Research Centre, Moscow, Russia.


Phaeochromocytomas are responsible for the development ACTH-ectopic syndrome in 5% cases. Less than 1% phaeochromocytoma accompanied by symptoms of ACTH-dependent hypercortisolism. We observed four cases of ACTH-secreting phaeochromocytoma during 2 years. All patients were women from 50 to 63 years. The disease duration ranged from 6 months to 5 years. The clinical picture presented the signs of hypercortisolism (matronism, hyperpigmentation of the skin, the redistribution of fatty tissue on the central type, and steroid myopathy) and hypercatecholaminaemia (arterial hypertension, tachycardia, shortness of breath, and frequent urination). Hormonal blood study found: morning ACTH 43.0–189.0 pg/ml (normal 7.0–66.0) evening 179.8–291.0 (normal 0–30.0), morning cortisol 962–4655 nmol/l (normal 123.0–626.0), evening 1256–2460 (normal 46.0–270.0), free cortisol in 24-h urine 3726.0–3828.0 nmol/day (normal 60.0–413.0); metanephrine in daily urine in three of four patients (638.0–1481.0 μg/day at the rate of 25.0–312.0) and normetanephrine (545.6–1553 μg/day at the rate of 35.0–445.0) (one patient had normal catecholamine levels). CT scans revealed tumors in the left adrenal glands sizes and density: 2.0×1.6×1.0 cm (27H), 2.7×3.0×4.6 cm (38H), and diameter 4.3 cm (density did not specified). In one case found two nodes: 1.8×2.3×2.5 and 2.0×2.3×2.5 cm (19H and 3H). Histological examination diagnosed three phaeochromocytoma combined with diffuse-nodular adrenal cortex hyperplasia and one mixed tumour (phaeochromocytoma and adenoma of the adrenal cortex). Immunohistochemical examination confirmed expression of ACTH in all cases. In all patients developed adrenal insufficiency and normalized levels of catecholamines after removal of adrenal tumors.

Conclusion: The diagnosis of ACTH-secreting phaeochromocytoma was established on the symptoms of hypercortisolism and hypercatecholaminaemia, excessive secretion of ACTH, cortisol and catecholamines in the blood and urine, the presence of neoplasms in the adrenal glands, the normalization of respective hormones after tumours removal, histological, and immuhistochemical confirmation of ACTH-secreting phaeochromocytomas.

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