Endocrine Abstracts

Cushing syndrome due to ectopic (adrenocorticotropic hormone) ACTH secretion (EAS) constitutes approximately 10% of Cushing’s syndrome (CS). In this group only in about 5% cases pheochromocytoma is the source of ACTH.

We present two patients with EAS by pheochromocytoma.

1. A 70 year-old woman with 3-months history of malaise, weakness, abdominal pain, loss of weight and appetite, hypertension and diabetes mellitus. One month earlier she had an episode of acute renal insufficiency after coronarography. Patient revealed mental confusion, cachexia, swelling of legs, skin with increased pigmentation, echymoses and petechiae. The laboratory data showed elevated leukocytosis, hyperglycemia, severe hypokalemia, and metabolic alkalosis, markedly elevated serum cortisol concentration and ACTH without cortisol suppression after dexamethasone. Measurements of Metanephrine and Normetanephrine in urine were increased. MRI and somatostatin receptor scintigraphy (SRS) disclosed 40 mm mass in right adrenal gland. After pharmacological treatment patient was laparoscopic operated. Histological examinations confirmed EAS and pheochromocytoma. Unfortunately in the 9-th day after surgery patient died because of ARDS syndrome.

2. A 61 year-old woman with 2-months history of weight gain, proximal myopathy, depressive disorders, abdominal pain, diabetes mellitus of recent onset and worseness of hypertension control. Patient had facial and leg edema, plethoric face, dermal and muscle atrophy and moderate central obesity. The laboratory data showed hyperglycemia, moderate hypokalemia, moderate leukocytosis, disturbed circadian cortisol rhythm and increased ACTH level. Measurements of Metanephrine and Normetanephrine in urine were increased. Abdominal CT scan and SRS revealed 30 mm mass in right adrenal gland and radiological signs suggested pheochromocytoma. After pharmacological treatment patient was successful laparoscopic operated, and all signs of CS regressed. Histological examinations confirmed EAS and pheochromocytoma.

EAS is difficult to diagnose. Other organs lesions make the prognosis worse.