Adrenocorticotropic Hormone Secreting Pheochromocytoma Underlying Glucocorticoid Induced Pheochromocytoma Crisis

Abstract

Context Pheochromocytomas are hormone secreting tumors of the medulla of the adrenal glands found in 0.1–0.5% of patients with hypertension. The vast majority of pheochromocytomas secrete catecholamines, but they have been occasionally shown to also secrete interleukins, calcitonin, testosterone, and in rare cases adrenocorticotropic hormone. Pheochromocytoma crisis is a life threatening event in which high levels of catecholamines cause a systemic reaction leading to organ failure. Case Description A 70-year-old man was admitted with acute myocardial ischemia following glucocorticoid administration as part of an endocrine workup for an adrenal mass. Cardiac catheterization disclosed patent coronary arteries and he was discharged. A year later he returned with similar angina-like chest pain. During hospitalization, he suffered additional events of chest pain, shortness of breath, and palpitations following administration of glucocorticoids as preparation for intravenous contrast administration. Throughout his admission, the patient demonstrated both signs of Cushing's syndrome and high catecholamine levels. Following stabilization of vital parameters and serum electrolytes, the adrenal mass was resected surgically and was found to harbor an adrenocorticotropic hormone secreting pheochromocytoma. This is the first documented case of adrenocorticotropic hormone secreting pheochromocytoma complicated by glucocorticoid induced pheochromocytoma crisis. Conclusion Care should be taken when administering high doses of glucocorticoids to patients with suspected pheochromocytoma, even in a patient with concomitant Cushing's syndrome.

Adrenocorticotropic hormone-secreting phaeochromocytoma as a cause of treatment-resistant hypertension and recurrent pulmonary emboli

“…Similar presentations of hypertension, refractory to treatment, with Cushingoid features have been reported previously. 2,6,7 Phaeochromocytoma was suspected by unilaterally elevated levels of methoxycatecholamines on AVS but confirmed through immunohistochemistry, similarly to a case described by Ramasamy et al 11 As previously reported, 82% of ACTH-secreting phaeochromocytomas occur in women, 61% in the left adrenal gland with a median age of 50 years at diagnosis and 83% prevalence of hypokalaemia, which is how our patient presented. 3,7 ACTH-induced hypercortisolism saturated mineralocorticoid receptors in our patient, causing low renin and hypokalaemia, but aldosterone was unusually elevated at presentation, likely explained by the paracrine effects of ACTH produced by the tumour.…”

“…1 Excess pituitary secretion of ACTH accounts for 80-90% of ACTHdependent cases whilst ectopic secretion of ACTH accounts for 10-20% with the commonest sources originating from bronchial neuroendocrine tumour, medullary thyroid cancer or small cell lung cancer. 2,3 An ACTH-secreting phaeochromocytoma was first reported in 1964 and thought to be rare but is now found to occur in 3-25% of cases with ectopic ACTH-secretion. [4][5][6] There are likely <100 cases of ACTH-secreting phaeochromocytoma reported worldwide.…”

“…3,7,8 Whilst hypertension appears to be the commonest presentation, occurring in 93% of cases, other patients have initially presented with hypokalaemia (83%), glucocorticoid-induced phaeochromocytoma crisis (18%) or rarely incidental adrenal adenoma. [2][3][4][5][6][7][8][9][10] Our patient presented with Cushing's syndrome and venous thromboembolism (VTE), with VTE occurring in 8% of patients with Cushing's syndrome pre-operatively. [4][5][6]8,11,12 She was referred to endocrinology, in Sweden, for suspected Cushing's syndrome.…”

A rare cause of severe Cushing’s syndrome

“…Cushing's syndrome occurs in 0.7-2.4 cases per million population per year (1). The majority (80%) of cases are ACTH driven, while 20% of the cases are ACTH independent, where the primary abnormality is in the adrenal gland (3). Around 80% of ACTH-dependent cases are estimated to arise from the pituitary gland (Cushing's disease) and the remainder is due to ectopic production of ACTH (4).…”

DIAGNOSIS OF ENDOCRINE DISEASE: Drug-induced endocrinopathies and diabetes: a combo-endocrinology overview

“…Dexamethasone and betamethasone, which have high glucocorticoid potency and long duration of action, are more likely to induce a pheochromocytoma crisis. Thus, in patients with adrenal incidentalomas, glucocorticoids should be avoided or administered cautiously especially if the suspicion for pheochromocytoma is high (208). However, if dexamethasone suppression test is considered necessary before ruling out pheochromocytoma, the endocrinologist should preferably proceed with the overnight dexamethasone suppression protocol, as no cases of pheochromocytoma crisis have been reported, as yet, with this dose (209).…”