Cushing's syndrome due to an ectopic ACTH‐secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production

Bethge, H.; Arlt, Wiebke; Zimmermann, U.; Klingelhöffer, G.; Wittenberg, Gregory P.; Saeger, Wolfgang; Allolio, Bruno

doi:10.1046/j.1365-2265.1999.00897.x

Cited by 22 publications

(9 citation statements)

References 25 publications

(47 reference statements)

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“…As for the origin of ectopic pituitary tumors including EAPA, several explanations have been proposed [21]. Pituitary cells could be deposited along the route of embryogenic development of the anterior pituitary, namely Rathke's pouch, and then the remnant tissue may develop into hormonally active adenomas in the ectopic regions such as sphenoid or cavernous sinus.…”

Section: Discussionmentioning

confidence: 99%

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An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

et al. 2004

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Abstract.A 32-year-old woman with an ectopic adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma (EAPA) is presented. She had rapidly gained weight and suffered recurrent facial acne for a few years but lacked the typical Cushingoid features. Endocrine examinations revealed that her plasma ACTH was markedly high (196 to 280 pg/ml) without showing normal circadian rhythm and failed to respond to corticotropin-releasing hormone stimulation. Her cortisol levels ranged from 22 to 30 mg/dl throughout observation but low doses (1 and 2 mg) of dexamethasone failed to suppress either ACTH or cortisol level. Magnetic resonance imaging study revealed a 3-cm mass occupying the sphenoidal sinus with partial enhancement by gadolinium, which was separated from the normal pituitary in the sella region. The tumor resected by transsphenoidal surgery was histologically diagnosed as an ACTH-producing pituitary adenoma. After surgery her weight gain and acne remitted in accordance with decreases in plasma ACTH. Analysis of patient plasma by gel filtration method revealed the existence of big ACTH molecules eluted with a peak of authentic 1-39 ACTH, suggesting that this biologically less-active ACTH might be the reason why overt features of Cushing's syndrome failed to develop in this case. Although EAPA is clinically rare in parasellar disorders, the presence of ectopic pituitary adenoma should be considered in such cases showing ACTH hypersecretion without typical Cushingoid features.

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Section: Discussionmentioning

confidence: 99%

“…1A and B). On her endocrine examinations, plasma ACTH level was markedly increased (196 to 280 pg/ml; normal, 9-52) but the serum cortisol level was not remarkable (23 to 27 mg/dl; normal [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]. Daily secretion of ACTH and cortisol lacked the normal circadian rhythm (Fig.…”

Section: Case Reportmentioning

confidence: 99%

An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

et al. 2004

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“…A limited number of ACTH-expressing intraabdominal [1-5] or intrathoracic [6-11] paragangliomas can be found in literature, however, to our knowledge, only two cases of ACTH-expressing nasal paragangliomas have been reported [12,13]. Furthermore, there have been four reports of ectopic ACTH-secreting pituitary adenomas, three of them localised in the sphenoidal sinus [14-16] and one intracavernously [17] as well as seven reports of ACTH-secreting olfactory neuroblastomas [18-24]. We present a rare case of severe hypercortisolemia due to ectopic ACTH secretion by a nasal paraganglioma.…”

Section: Introductionmentioning

confidence: 99%

Hypercortisolaemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma: a case report and review of the literature

et al. 2013

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BackgroundAdrenocorticotropic hormone-producing extraadrenal paragangliomas are extremely rare. We present a case of severe hypercortisolemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma.Case presentationA 70-year-old Caucasian woman was emergently admitted to our department with supraventricular tachycardia, oedema of face and extremities and hypertensive crisis. Initial laboratory evaluation revealed severe hypokalemia and hyperglycemia without ketoacidosis, although no diabetes mellitus was previously known. Computed tomography revealed a large tumor obliterating the left paranasal sinus and a left-sided adrenal mass. After cardiovascular stabilisation, a thorough hormonal assessment was performed revealing marked adrenocorticotropic hormone-dependent hypercortisolism. Due to the presence of a cardiac pacemaker magnetic resonance imaging of the hypophysis was not possible. [68Ga-DOTA]-TATE-Positron-Emission-Tomography was performed, showing somatostatin-receptor expression of the paranasal lesion but not of the adrenal lesion or the hypophysis. The paranasal tumor was resected and found to be an adrenocorticotropic hormone-producing paraganglioma of low-proliferative rate. Postoperatively the patient became normokaliaemic, normoglycemic and normotensive without further need for medication. Genetic testing showed no mutation of the succinatdehydrogenase subunit B- and D genes, thus excluding hereditary paragangliosis.ConclusionDetection of the adrenocorticotropic hormone source in Cushing’s syndrome can prove extremely challenging, especially when commonly used imaging modalities are unavailable or inconclusive. The present case was further complicated by the simultaneous detection of two tumorous lesions of initially unclear biochemical behaviour. In such cases, novel diagnostic tools - such as somatostatin-receptor imaging - can prove useful in localising hormonally active neuroendocrine tissue. The clinical aspects of the case are discussed and relevant literature is reviewed.

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“…For example, incorrect placement of catheters, anatomical abnormalities, the rare occurrence of a hypoplastic IPS (10), unusual location of the adenoma (29,30), anomalous venous drainage or dilution of the petrosal venous blood (31,32) have been reported as possible causes in some of the false-negative cases. It is most unlikely that such settings could be affected by the enhanced ACTH release produced by the combined regime.…”

Section: Discussionmentioning

confidence: 99%

The Application of the Combined Corticotropin-Releasing Hormone plus Desmopressin Stimulation during Petrosal Sinus Sampling Is Both Sensitive and Specific in Differentiating Patients with Cushing’s Disease from Patients with the Occult Ectopic Adrenocorticotropin Syndrome

Tsagarakis

Vassiliadi

Kaskarelis

et al. 2007

The Journal of Clinical Endocrinology &Amp; Metabolism

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Context:Although bilateral inferior petrosal sinus sampling (BI-PSS) with CRH stimulation is the most accurate procedure for the differential diagnosis of ACTH-dependent Cushing's syndrome (CS), 4 -15% of patients with Cushing's disease (CD) fail to demonstrate diagnostic gradients. Preliminary data suggest that a more potent stimulation by the combined administration of CRH plus desmopressin during BIPSS may provide some diagnostic advantage. A crucial issue, however, is whether such an amplified stimulation may affect the specificity of the procedure, and this was the main aim of the present study. Objective:We investigated the diagnostic accuracy of BIPSS performed by CRH plus desmopressin stimulation. Design and Setting:A retrospective analysis was conducted at a single tertiary care center. Participants:Fifty-four patients were admitted for the investigation of ACTH-dependent CS. CD was diagnosed in 47 patients; occult ectopic ACTH syndrome (oEAS) was histologically confirmed in seven patients. Intervention(s):All patients underwent BIPSS with CRH plus desmopressin administration. Additional noninvasive tests included CRH test, high-dose dexamethasone suppression test, desmopressin test, and pituitary magnetic resonance imaging. Main Outcome Measures:Gradients of inferior petrosal sinus (IPS) to peripheral (IPS/P) ACTH were calculated before and after stimulation with CRH plus desmopressin. Results:The sensitivity for a basal IPS/P gradient greater than 2 was 61.7%, with 100% specificity and a diagnostic accuracy of 66.7%. After stimulation with CRH plus desmopressin, receiver operating characteristic (ROC) curve analysis showed that a cutoff gradient of more than 2 offers the best test performance. In total, 46 of 47 patients with CD had an IPS/P gradient greater than 2, but none of the patients with oEAS, resulting in a sensitivity of 97.9%. The specificity was 100%, diagnostic accuracy was 98.2%, and the positive and negative predictive values were 100 and 87.5%, respectively. A subgroup of 18 patients (16 with CD and two with oEAS) had contradictory responses to routine tests with CRH and/or high-dose dexamethasone suppression test; sensitivity, specificity, and accuracy of BIPSS in this subgroup were 100%. Conclusions:The application of a combined stimulation with CRH plus desmopressin during BIPSS is associated with a high sensitivity but no loss of specificity. (J Clin Endocrinol Metab

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Cushing's syndrome due to an ectopic ACTH‐secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production

Cited by 22 publications

References 25 publications

An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

Hypercortisolaemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma: a case report and review of the literature

The Application of the Combined Corticotropin-Releasing Hormone plus Desmopressin Stimulation during Petrosal Sinus Sampling Is Both Sensitive and Specific in Differentiating Patients with Cushing’s Disease from Patients with the Occult Ectopic Adrenocorticotropin Syndrome

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