Phaeochromocytoma and <scp>ACTH</scp>‐dependent cushing's syndrome: tumour crf secretion can mimic pituitary cushing's disease

Lois, KB; Santhakumar, Anjali; Suresh, V.; Mathew, Shaju; Long, Anna; Johnson, SJ; Peaston, RT; Neely, Rdg; Richardson, DL; Graham, Janine; Lennard, Twj; Bliss, Richard; Miller, Margaret A.; Ball, Stephen; Pearce, Simon H. S.; Woods,; Quinton, Richard

doi:10.1111/cen.12960

Cited by 16 publications

(17 citation statements)

References 44 publications

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“…Post-operative recovery from the clinical symptoms and elevated cortisol levels is much faster in patients with ectopic CRH syndrome than in those with ectopic ACTH syndrome [5, 8]. In the present case, the rapid postoperative recovery of clinical features, elevated serum ACTH, potassium and glucose levels, and short-term application of prednisone supported the classification of the pheochromocytoma as a CRH-secreting tumor, with ACTH secreted from the pituitary gland.…”

Section: Discussionsupporting

confidence: 61%

“…Quinton et al reported three patients with ACTH-dependent Cushing’s syndrome; in two cases, immunostaining showed reactivity for CRH instead of ACTH, supporting CRH (or related peptide) -secreting pheochromocytoma [8]. In the current case, the patient’s rapidly deteriorating condition did not allow us to perform IPSS and adrenal vein sampling.…”

Section: Discussionmentioning

confidence: 65%

“…Studies have reported that EAS and ectopic CRH syndrome have different effects on the hypothalamic-pituitary-adrenal axis [8]. Post-operative recovery from the clinical symptoms and elevated cortisol levels is much faster in patients with ectopic CRH syndrome than in those with ectopic ACTH syndrome [5, 8].…”

Section: Discussionmentioning

confidence: 99%

“…The first case of isolated ectopic CRH-secreting pheochromocytoma was reported in 1999 [4]. To date, six cases of isolated ectopic CRH-secreting pheochromocytoma and three cases of ectopic ACTH/CRH co-secreting pheochromocytoma have been reported [4, 5, 8–13]. Usually, a pheochromocytoma produces catecholamines and causes symptoms such as hypertension [14].…”

Section: Discussionmentioning

confidence: 99%

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An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

et al. 2018

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BackgroundPheochromocytoma, especially for noncatecholamine-secreting pheochromocytoma, is an extremely rare cause of ectopic corticotrophin-releasing hormone (CRH) syndrome.Case presentationA 27-year-old Chinese woman was administered dexamethasone for a skin allergy, but her general condition rapidly deteriorated over a month. She was subsequently hospitalized for typical clinical features of Cushing’s syndrome. Endocrinological investigation confirmed severe hypercortisolism along with elevated plasma adrenocorticotropin hormone (ACTH). However, magnetic resonance imaging (MRI) revealed no pituitary adenoma. Abdominal contrast-enhanced computed tomography (CT) revealed a 6.5 cm heterogeneous right adrenal mass with mildly contrast enhancement. The tumor was found during a routine physical check-up at a local hospital 16 months ago; however, the patient did not have any symptoms and did not seek further medical attention at that time. Laparoscopic resection of the right adrenal tumor led to a rapid remission of Cushing’s syndrome. Based on pathological findings and the presence of normal catecholamine metabolites in her serum and urine, the patient was diagnosed with noncatecholamine-secreting pheochromocytoma. Immunohistochemical staining of the adrenal tumor revealed positive staining for CRH and negative staining for ACTH.ConclusionsThis is an extremely rare case of ectopic CRH syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma. Both ectopic ACTH syndrome and ectopic CRH syndrome should be considered in patients presenting with ACTH-dependent Cushing’s syndrome caused by extrapituitary diseases.

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Section: Discussionsupporting

confidence: 61%

Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

et al. 2018

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“…Furthermore, they have been shown to affect the results of dynamic tests and may generate false positive results during BIPSS (5, 6, 8, 10, 12, 17, 24, 25, 26, 27). CRH secretion induces diffuse hyperplasia of pituitary corticotroph cells which then oversecrete ACTH but may remain responsive to both exogenous dexamethasone and CRH.…”

Section: Discussionmentioning

confidence: 99%

A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing's disease

Streuli

Krull

Brändle

et al. 2017

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing’s syndrome and only a few cases have been reported in the literature. Differentiating between Cushing’s disease and ectopic Cushing’s syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing’s disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS). We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing’s syndrome caused by an ACTH/CRH co-secreting midgut NET. Both high-dose dexamethasone testing and BIPSS suggested Cushing’s disease. However, the clinical presentation with a rather rapid onset of cushingoid features, hyperpigmentation and hypokalemia led to the consideration of ectopic ACTH/CRH-secretion and prompted a further workup. Computed tomography (CT) of the abdomen revealed a cecal mass which was identified as a predominantly CRH-secreting neuroendocrine tumor. To the best of our knowledge, this is the first reported case of an ACTH/CRH co-secreting tumor of the cecum presenting with biochemical features suggestive of Cushing’s disease.Learning points:The discrimination between a Cushing’s disease and ectopic Cushing’s syndrome is challenging and has many caveats.Ectopic ACTH/CRH co-secreting tumors are very rare.Dynamic tests as well as BIPSS may be compatible with Cushing’s disease in ectopic CRH-secretion.High levels of CRH may induce hyperplasia of the corticotroph cells in the pituitary. This could be the cause of a preserved pituitary response to dexamethasone and CRH.Clinical features of ACTH-dependent hypercortisolism with rapid development of Cushing’s syndrome, hyperpigmentation, high circulating levels of cortisol with associated hypokalemia, peripheral edema and proximal myopathy should be a warning flag of ectopic Cushing’s syndrome and lead to further investigations.

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Adrenal Neoplasms

Stewart

Story

2017

Textbook of Uncommon Cancer

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Phaeochromocytoma and ACTH‐dependent cushing's syndrome: tumour crf secretion can mimic pituitary cushing's disease

Cited by 16 publications

References 44 publications

An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing's disease

Adrenal Neoplasms

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