Severe Ectopic Cushing’s Syndrome Due to ACTH-Secreting Pheochromocytoma

Abstract

We report a new case of ectopic Cushing's syndrome caused by an ACTH-producing pheochromocytoma. A 55-yearold woman presented with a history of severe proximal muscle weakness, polyuria, progressive virilization, anxiety, dyspnea on exercise, difficult to treat hypertension, and type 2 diabetes mellitus since 4 months. The laboratory data demonstrated ACTH-dependent hypercortisolism. The abdominal computed tomography scan showed a 30 mm welldefined mass in the left adrenal gland suggestive for pheochromocytoma. The adrenal veins were sampled, with intraprocedural cortisol measurement, to dosing selective ACTH and cathecolamines. The results established clearly the left adrenal gland as the source of ACTH overproduction. A left sided adrenalectomy was performed with subsequent resolution of Cushing's syndrome. The patient was discharged in good clinical condition.

Bowel perforation complicating an ACTH-secreting phaeochromocytoma

“…Since 1977, there have been two likely cases of ASP; however, death occurred before fulfilment of the diagnostic criteria – myocardial infarction in a 67-year-old female and septic cerebral emboli in a 28-year-old female ( 13 , 16 ). The 29 surviving cases had multiple complications including cardiac arrest, pneumonia with respiratory failure, hypertensive encephalopathy, psychosis, cerebellar infarction, pulmonary embolus, haemorrhagic shock and sepsis ( 3 , 15 , 17 , 18 , 19 , 20 ). This highlights the morbidity associated with elevated cortisol and catecholamines and the need for prompt normalisation.…”

ACTH ectopic syndrome caused by corticomedullary mixed tumor

“…Возможно также паракринное взаимодействие феохромоцитомы и коры надпочеч-ника, приводящее к образованию диффузно-узло-вой гиперплазии коры и повышению выработки кортизола. [13]. Описано несколько случаев фео-хромоцитомы и смешанно-клеточной кортико-ме-дуллярной опухоли, ассоциированой с «пре-Кушинг-синдромом», в отсутствие повышения уровня АКТГ в крови [14][15][16].…”

Adrenal incidentaloma: A case of pheochromocytoma with sub-clinical Cushing′s syndrome

“…Pheochromocytoma may secrete various substances such as somatostatin, enkephalines, calcitonin, vasoactive intenstinal peptide, neuropeptide-Y, renin, ACTH, parathyroid hormone, erythropoietin, adrenomedullin and dihydroxyphenylalanine (DOPA) in addition to catecholamines. [ 2 ] There are about thirty cases reported in the literature of Cushing's syndrome due to ACTH-secreting pheochromocytoma. [ 2 ] In some of these cases, there was pre-dominance of hyperpigmentation, hypokalemic alkalosis and evidence of contralateral adrenal gland hyperplasia on imaging, but these features were absent in our case.…”

“…[ 2 ] There are about thirty cases reported in the literature of Cushing's syndrome due to ACTH-secreting pheochromocytoma. [ 2 ] In some of these cases, there was pre-dominance of hyperpigmentation, hypokalemic alkalosis and evidence of contralateral adrenal gland hyperplasia on imaging, but these features were absent in our case.…”

“…[ 1 ] A combination of pheochromocytoma and Cushing's syndrome due to pathology in same adrenal gland is extremely rare. There are few case reports suggesting the different etiologies for this association, namely, pheochromocytoma secreting adrenocorticotropin hormone (ACTH)[ 2 ] or its precursors,[ 3 ] corticomedullary mixed tumors[ 4 ] and focal adrenocortical hyperplasia. [ 5 ]…”