Cushing's syndrome presenting the coexistence of a pituitary corticotrophic cell hyperplasia and a unilateral functional adrenal adenoma

1985 ◽  
Vol 110 (3) ◽  
pp. 302-307 ◽  
Author(s):  
Hajime Watanobe ◽  
Takahiko Kawagishi ◽  
Yuichi Hirai ◽  
Tatsuro Sato ◽  
Masahiro Tsutsui ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Anterior Pituitary ◽  
Unusual Case ◽  
Similar Case ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Cell Hyperplasia ◽  
Adrenocortical Hyperplasia ◽  
Acth Concentration

Abstract. A very unusual case of Cushing's syndrome is presented. Most of the preoperative biochemical and radiological examinations were compatible with Cushing's syndrome owing to a right adrenal adenoma. Exceptional findings include normal concentrations of adrenocorticotrophin (ACTH) in plasma as well as a disturbance of its circadian rhythmicity and a significant adrenocortical responsiveness to exogenous ACTH. Secretory patterns of ACTH did not change even after right adrenalectomy. Studies in vitro revealed that the adenoma itself, but not the surrounding normal adrenal, was the source of cortisol secreted in response to ACTH. Post mortem examinations disclosed unexpectedly a hormonally inactive left adrenal adenoma and a focal hyperplastic lesion of the anterior pituitary with an ACTH concentration 53 times higher than that of the remaining tissue of the gland. It is a possibility that this case may have represented a transition between pituitary-dependent adrenocortical hyperplasia and adrenal adenoma to this date reported in only one similar case.

scholarly journals Combined Primary Aldosteronism and Preclinical Cushing's Syndrome: An Unusual Case Presentation of Adrenal Adenoma.

2001 ◽  
Vol 24 (6) ◽  
pp. 723-726 ◽  
Author(s):  
Tadaaki HONDA ◽  
Tetsuya NAKAMURA ◽  
Yuichiro SAITO ◽  
Yoshio OHYAMA ◽  
Hiroyuki SUMINO ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Primary Aldosteronism ◽  
Unusual Case ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Case Presentation

scholarly journals cAMP signaling in cortisol-producing adrenal adenoma

2015 ◽  
Vol 173 (4) ◽  
pp. M99-M106 ◽  
Author(s):  
Davide Calebiro ◽  
Guido Di Dalmazi ◽  
Kerstin Bathon ◽  
Cristina L Ronchi ◽  
Felix Beuschlein
Keyword(s):  
Signaling Pathway ◽  
Cushing’S Syndrome ◽  
Cell Function ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Camp Signaling ◽  
Common Finding ◽  
Adrenocortical Adenomas ◽  
Camp Signaling Pathway

The cAMP signaling pathway is one of the major players in the regulation of growth and hormonal secretion in adrenocortical cells. Although its role in the pathogenesis of adrenocortical hyperplasia associated with Cushing's syndrome has been clarified, a clear involvement of the cAMP signaling pathway and of one of its major downstream effectors, the protein kinase A (PKA), in sporadic adrenocortical adenomas remained elusive until recently. During the last year, a report by our group and three additional independent groups showed that somatic mutations of PRKACA, the gene coding for the catalytic subunit α of PKA, are a common genetic alteration in patients with Cushing's syndrome due to adrenal adenomas, occurring in 35–65% of the patients. In vitro studies revealed that those mutations are able to disrupt the association between catalytic and regulatory subunits of PKA, leading to a cAMP-independent activity of the enzyme. Despite somatic PRKACA mutations being a common finding in patients with clinically manifest Cushing's syndrome, the pathogenesis of adrenocortical adenomas associated with subclinical hypercortisolism seems to rely on a different molecular background. In this review, the role of cAMP/PKA signaling in the regulation of adrenocortical cell function and its alterations in cortisol-producing adrenocortical adenomas will be summarized, with particular focus on recent developments.

Incidentally discovered ACTH-dependent adrenal adenoma presenting as 'Pre-Cushing's syndrome'

1986 ◽  
Vol 111 (1) ◽  
pp. 89-92 ◽  
Author(s):  
U. Bogner ◽  
U. Eggens ◽  
J. Hensen ◽  
W. Oelkers
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Mass ◽  
Adrenal Adenoma ◽  
Clinical Signs ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Tumour ◽  
Urinary Cortisol

Abstract. An adrenal tumour was incidentally discovered with no clinical signs of Cushing's syndrome. The endocrine evaluation revealed the unique hormonal constellation of an increased urinary cortisol excretion rate, unequivocal suppressibility of plasma and urinary cortisol by dexamethasone, but only to a residual level in the low normal range which probably reflected ACTH-independent 'autonomous' cortisol secretion. After removal of the adrenal mass, urinary cortisol secretion and dexamethasone suppressibility were normalized. In vitro, the tumour cells were as sensitive towards ACTH as 'normal' human adrenal cells, but showed a reduced cortisol production rate per cell. We suppose that the adrenal mass participated in the diurnal rhythm of ACTH-mediated cortisol secretion in vivo, which resulted in an increased cortisol secretion. During the night, when ACTH levels were low, the cortisol production decreased and the hormone levels were probably too low to suppress ACTH. We regard the hormonal findings in our patients as 'Pre-Cushing's syndrome', although the absence of clinical features of Cushing's syndrome remains unclear. We suggest that every patient with an incidentally discovered adrenal mass should have an endocrinological evaluation because the results may help to decide whether or not the adrenal tumour should be removed.

THE EXCRETION OF INDIVIDUAL 17-OXOSTEROIDS IN CUSHING'S SYNDROME

1961 ◽  
Vol 23 (2) ◽  
pp. 119-127 ◽  
Author(s):  
V. H. T. JAMES
Keyword(s):  
Urinary Excretion ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
High Ratio ◽  
Hepatic Enzymes ◽  
Normal Range ◽  
Adrenal Androgen ◽  
Adrenocortical Hyperplasia ◽  
Secretion Rates

SUMMARY The urinary excretion levels of individual 17-oxosteroids in eight patients with Cushing's syndrome have been determined quantitatively. Using these values approximate secretion rates of cortisol and 11β-hydroxyandrostenedione were calculated and found to be elevated above the normal range. The excretion values for 11-deoxy-17-oxosteroids were highly variable, being below normal, normal or elevated in different patients. The excretion patterns in adrenocortical hyperplasia and carcinoma showed no characteristic differences; however, the patients with adrenal adenoma showed steroid excretions which suggested that these tumours secreted cortisol almost exclusively, together with small quantities of 11β-hydroxyandrostenedione, and minimal amounts of 11-deoxy-C19-steroids. The majority of patients excreted aetiocholanolone and androsterone in an abnormally high ratio; this also occurred after administration of androst-4-ene-3:17-dione, and it is suggested that in Cushing's syndrome the hepatic enzymes preferentially reduce adrenal androgen to metabolites with the 5β configuration.

scholarly journals Food-Dependent Cushing’s Syndrome: Possible Involvement of Leptin in Cortisol Hypersecretion*

1999 ◽  
Vol 84 (10) ◽  
pp. 3817-3822 ◽  
Author(s):  
François P. Pralong ◽  
Fulgencio Gomez ◽  
Louis Guillou ◽  
François Mosimann ◽  
Sebastiano Franscella ◽  
...  
Keyword(s):  
Food Intake ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Hyperplasia ◽  
Adrenal Cells ◽  
Patient Reported ◽  
Stimulation Of

Abstract Stimulation of cortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing’s syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food-dependent Cushing’s syndrome in a patient with bilateral macronodular adrenal hyperplasia. In this patient we were able to confirm a paradoxical stimulation of cortisol secretion by GIP in vivo as well as in vitro on dispersed tumor adrenal cells obtained at surgery. In addition to GIP, in vitro stimulation of these cultured tumor adrenal cells with leptin, the secreted product of the adipocyte, induced cortisol secretion. By comparison, no such stimulation was observed in vitro in adrenal cells obtained from another patient with bilateral macronodular adrenal hyperplasia and Cushing’s syndrome that did not depend on food intake, in tumor cells obtained from a solitary cortisol-secreting adrenal adenoma, and in normal human adrenocortical cells. These results demonstrate that as in previously described cases of food-dependent Cushing’s syndrome, GIP stimulated cortisol secretion from the adrenals of the patient reported here. Therefore, they indicate that such a paradoxical response probably represents the hallmark of this rare condition. In addition, they suggest that leptin, which normally inhibits stimulated cortisol secretion in humans, participated in cortisol hypersecretion in this case. Further studies in other cases of food-dependent Cushing’s syndrome, however, will be necessary to better ascertain the pathophysiological significance of this finding.

scholarly journals An Unusual Case of Cushing’s Syndrome: Coexistence of Functional Pituitary and Adrenal Adenoma

2017 ◽  
Vol 55 (1) ◽  
pp. 61-63
Author(s):  
Muzaffer İlhan ◽  
Özcan Karaman ◽  
İrem Yasin Çetin ◽  
Nur Büyükpınarbaşılı ◽  
Jamshid Hamdard ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Unusual Case ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome

scholarly journals Unilateral nodular adrenocortical hyperplasia presenting as Cushing’s syndrome: a case report

2021 ◽  
Vol 12 (1) ◽  
pp. 78-82
Author(s):  
Lubna Naznin ◽  
Susane Giti ◽  
SK Md Jaynul Islam ◽  
Khandaker Rokshana Akhter ◽  
Shamoli Yasmin ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Histopathological Examination ◽  
Case Reports ◽  
Adrenal Adenoma ◽  
Final Diagnosis ◽  
Cushing's Syndrome ◽  
Uncontrolled Hypertension ◽  
Secondary Hypothyroidism ◽  
Adrenocortical Hyperplasia

Cushing syndrome results from chronic exposure to excess cortisol. Nodular adrenal hyperplasia is usually bilateral and has only a few case reports of unilateral presentation. Biochemically it is presented as hyperaldosteronism or as Cushing’s syndrome. Here, we are reporting a 17-year-old female presenting with weight gain over 5 months and uncontrolled hypertension. Biochemically she was found to have diabetes mellitus, secondary hypothyroidism and hypogonadotrophic hypogonadism due to Cushing’s syndrome of adrenal origin. Unilateral adrenal adenoma/hyperplasia in right adrenal gland was evident by radiology. Histopathological examination was done after laparoscopic adrenalectomy showed nodular adrenocortical hyperplasia in right adrenal mass. Following surgery, clinical features of the patient improved notably. Cushing syndrome due to unilateral nodular adrenocortical hyperplasia is a rare entity. Biochemical evaluation of hypothalamo-pituitary-adrenal axis, radiological evidence and histopathology are the important armaments can guide to final diagnosis. BIRDEM Med J 2022; 12(1): 78-82

scholarly journals Long term control of hypercortisolism with fluconazole: case report and in vitro studies

2006 ◽  
Vol 154 (4) ◽  
pp. 519-524 ◽  
Author(s):  
Michaela Riedl ◽  
Christina Maier ◽  
Georg Zettinig ◽  
Peter Nowotny ◽  
Wolfgang Schima ◽  
...  
Keyword(s):  
Case Report ◽  
Cell Line ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Unilateral Adrenalectomy ◽  
Cortisol Excretion ◽  
Glucocorticoid Production

Objectives: To evaluate the efficacy of fluconazole as an alternative treatment for controlling hypercortisolism in Cushing’s syndrome and to determine its effect on glucocorticoid production in vitro. Design: Case report and in vitro study in a University Clinic. Case: An 83 year old patient presented with recurrence of Cushing’s syndrome due to pulmonary metastases three years after unilateral adrenalectomy. During a near fatal episode of sepsis she was started on fluconazole 200 mg/day intravenously which normalised cortisol excretion. The therapy was continued orally for 18 months. Upon temporary discontinuation and reintroduction of treatment, cortisol levels increased and normalized, respectively. At month 16, fluconazole had to be increased to a dose of 400 mg/day to keep cortisol excretion in the normal range. Disease progression was slow and no side effects occurred. In vitro results: Fluconazole in a concentration of 500 μM nearly abolished corticosterone production over 24 h from the adrenal adenoma cell line Y-1 (8.6 ± 0.5% compared with control, P < 0.0001) and significantly reduced corticosterone production in concentrations of 50 μM (48.3 ± 1.9% vs. control, P < 0.0001) and 5 μM (80.5 ± 8.5% vs. control, P < 0.05). Conclusion: These results demonstrate for the first time that fluconazole normalises cortisol concentrations in vivo in a patient with Cushing’s syndrome with adrenal carcinoma and inhibit glucocorticoid production in vitro in a cell line. Thus, fluconazole might be useful in controlling glucocorticoid excess in Cushing’s syndrome and because of its lower toxicity might be preferable to ketoconazole.

ACTH-lndependent Bilateral Macronodular Adrenocortical Hyperplasia Caused Cushing’s Syndrome

1997 ◽  
Vol 58 (4) ◽  
pp. 259-262 ◽  
Author(s):  
Akira Miyajima ◽  
Jun Nakashima ◽  
Masaaki Tachibana ◽  
Shim Baba ◽  
Kaoru Nakamura ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenocortical Hyperplasia
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