Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Gabi, Jenan; Milhem, Maali; Tovar, Yara E.; Karem, Emhemmid; Gabi, Alaa; Khthir, Rodhan

doi:10.1210/js.2018-00086

Cited by 36 publications

(62 citation statements)

References 51 publications

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“…Our patient did not have any evidence of Cushing's syndrome when he was first diagnosed with a phaeochromocytoma, but developed severe Cushing's syndrome within weeks. A recent literature review by Gabi et al confirmed that rapid onset of hypercortisolism appears to be a feature of ACTH-secreting phaeochromocytomas, unlike the insidious onset of other forms of ACTH-dependent Cushing's syndrome (1). Like our patient, the majority of patients had severe Cushingoid symptoms due to the circulating high levels of ACTH and cortisol.…”

Section: Discussionsupporting

confidence: 71%

“…Like our patient, the majority of patients had severe Cushingoid symptoms due to the circulating high levels of ACTH and cortisol. Severe hypokalaemia is a feature reported in 95% of those cases (1). High circulating levels of cortisol exhibit mineralocorticoid activity after saturation of the 11ß-hydroxysteroid dehydrogenase type 2, the enzyme responsible for its metabolism, thus causing hypokalaemia (6) .…”

Section: Discussionmentioning

confidence: 99%

“…Ectopic adrenocorticotropic hormone (ACTH) production is a rare cause of Cushing's syndrome and is usually seen in the context of small cell lung cancer, bronchial carcinoid or medullary thyroid cancer (1). Phaeochromocytoma is rarely associated with ectopic ACTH production (2).…”

Section: Introductionmentioning

confidence: 99%

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A rare cause of severe Cushing’s syndrome

Zaman

Patel

Glynne³

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Ectopic adrenocorticotropic hormone (ACTH) production is an uncommon cause of Cushing’s syndrome and, rarely, the source can be a phaeochromocytoma. A 55-year-old man presented following an episode of presumed gastroenteritis with vomiting and general malaise. Further episodes of diarrhoea, joint pains and palpitations followed. On examination, he was hypertensive with no clinical features to suggest hypercortisolaemia. He was subsequently found to have raised plasma normetanephrines of 3.98 nmol/L (NR <0.71) and metanephrines of 0.69 nmol/L (NR <0.36). An adrenal CT showed a 3.8 cm right adrenal nodule, which was not MIBG-avid but was clinically and biochemically consistent with a phaeochromocytoma. He was started on alpha blockade and referred for right adrenalectomy. Four weeks later, on the day of admission for adrenalectomy, profound hypokalaemia was noted (serum potassium 2.0 mmol/L) with non-specific ST-segment ECG changes. He was also diagnosed with new-onset diabetes mellitus (capillary blood glucose of 28 mmol/L). He reported to have gained weight and his skin had become darker over the course of the last 4 weeks. Given these findings, he underwent overnight dexamethasone suppression testing, which showed a non-suppressed serum cortisol of 1099 nmol/L. Baseline serum ACTH was 273 ng/L. A preliminary diagnosis of ectopic ACTH secretion from the known right-sided phaeochromocytoma was made and he was started on metyrapone and insulin. Surgery was postponed for 4 weeks. Following uncomplicated laparoscopic adrenalectomy, the patient recovered with full resolution of symptoms. Learning points: Phaeochromocytomas are a rare source of ectopic ACTH secretion. A high clinical index of suspicion is therefore required to make the diagnosis. Ectopic ACTH secretion from a phaeochromocytoma can rapidly progress to severe Cushing’s syndrome, thus complicating tumour removal. Removal of the primary tumour often leads to full recovery. The limited literature suggests that the presence of ectopic Cushing’s syndrome does not appear to have any long-term prognostic implications.

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

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A rare cause of severe Cushing’s syndrome

Zaman

Patel

Glynne³

et al. 2020

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…Pheochromocytoma/paraganglioma: These catecholamine-secreting endocrine tumors are a rare cause of EAS, and are responsible for less than 5% of all reported EAS cases (56,57,58,59). They are mostly unilateral, localized and retroperitoneal (18,51,52,53,54).…”

Section: (C) Abdominal and Retroperitoneal Netsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young

Haissaguerre

Viera-Pinto

et al. 2020

European Journal of Endocrinology

158

186

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Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.

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“…PPGLs are more frequent in certain groups, for example, in patients with adrenal incidentalomas with 0.6% to 4.2% being affected but is otherwise generally rare . Most PPGLs are nowadays diagnosed due to an incidentaloma, then due to catecholamine excess symptoms and finally because of screening in a previously known familial syndrome (eg, multiple endocrine neoplasia type 2, von Hippel Lindau syndrome, neurofibromatosis type 1, and mutations in succinate dehydrogenase B, C, and D) Cushing's syndrome due to ectopic ACTH‐production from a PPGL can occasionally occur, and thus all adrenal tumors should have a 1 mg overnight dexamethasone suppression test to exclude cortisol excess . Sometimes an adrenal medullary hyperplasia may be the culprit of catecholamine excess …”

Section: Introductionmentioning

confidence: 99%

Clinical features, complications, and outcomes of exogenous and endogenous catecholamine‐triggered Takotsubo syndrome: A systematic review and meta‐analysis of 156 published cases

Y‐Hassan

Falhammar

2020

Clinical Cardiology

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Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger Takotsubo syndrome (TS). A systematic search of PubMed/MEDLINE identified 156 patients with catecholamine-induced TS up to December 2017. Data were compared within the catecholamine-induced TS cohort, but some comparisons were also done to a previously published large all-TS cohort (n = 1750). The mean age was 46.4 ± 16.4 years (72.3% women). The clinical presentation was dramatic with high complication rates in (68.2%, n = 103; multiple complications 34.6%, n = 54). The most common TS ballooning pattern was apical or mid-apical (45.2%, n = 69), followed by basal pattern (28.8%, n = 45), global pattern (16.0%, n = 25), midventricular (8.3%, n = 13), focal (0.6%, n = 1), and unidentified pattern (1.9%, n = 3).There was an increase in the prevalence of apical sparing ballooning pattern compared to all-TS population (37.7% vs 18.3%, P < .00001). Higher complication rates were observed in TS with global ballooning pattern compared to apical ballooning pattern (23/25, 92% vs 38/65, 58.5%; P = .0022). Higher complication rates were observed in patients with age < 50 years than patients >50 years (73/92, 79.3% vs 29/56, 51.8%, P = 0.0009). Recurrence occurred exclusively in patients with PPGLinduced TS (18/107 patients, 16.8%). PPGL-induced TS was characterized by more global ballooning's pattern (22/104, 21.2% vs 3/49, 6.1%, P = 0.02), and lower left ventricular ejection fraction (25.54 ± 11.3 vs 31.82 ± 9.93, P = 0.0072) compared to exogenous catecholamine-induced TS. In conclusion, catecholamine-induced TS was characterized by a dramatic clinical presentation with extensive left ventricular dysfunction, and high complication rate. K E Y W O R D S broken heart syndrome, epinephrine, myocardial stunning, norepinephrine, paraganglioma, pheochromocytoma, Takotsubo

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Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Cited by 36 publications

References 51 publications

A rare cause of severe Cushing’s syndrome

A rare cause of severe Cushing’s syndrome

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Clinical features, complications, and outcomes of exogenous and endogenous catecholamine‐triggered Takotsubo syndrome: A systematic review and meta‐analysis of 156 published cases

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