scholarly journals Ectopic Cushing’s syndrome due to retroperitoneal ACTH-producing paragangliomas

2016 ◽  
Vol 10 (9-10) ◽  
pp. 320 ◽  
Author(s):  
Fan Chen ◽  
Xiangyu Wang ◽  
Yang Wang ◽  
Hui Meng ◽  
Yaofeng Zhu ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Chromaffin Cells ◽  
Adrenocorticotropic Hormone ◽  
Left Kidney ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Rare Tumours ◽  
Bilateral Adrenal Hyperplasia ◽  
Laboratory Examinations ◽  
Adrenal Chromaffin

Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing’s syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup.

scholarly journals MON-LB44 Cushing’s Syndrome and Illegal Receptors

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Caroline Poku ◽  
Randa Sharag Eldin ◽  
Abubakr Hassab Elrasoul Babiker Mohamed ◽  
Sadiq Sobia
Keyword(s):  
Cushing’S Syndrome ◽  
Hormone Receptors ◽  
Age Distribution ◽  
Cushing's Syndrome ◽  
Endocrine Surgery ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Production Studies ◽  
Bilateral Adrenal Hyperplasia ◽  
Adrenal Masses

Abstract Case presentation: A 48-year-old female with HTN presented to the endocrinology clinic for the evaluation of incidental bilateral adrenal masses noted on chest CT for dyspnea workup. At the time of the presentation, she reported generalized fatigue, significant weight gain in the past year and shortness of breath. Her physical exam was remarkable for central obesity. Lab work showed elevated cortisol after 1 mg dexamethasone suppression test x 2 and elevated 24- hour urine cortisol. Plasma free metanephrine levels and aldosterone/ renin ratio were normal. MRI abdomen was done and showed bilateral adrenal masses (left: 5.6 cm, right: 3.2 cm). Patient was diagnosed with Cushing’s syndrome secondary to primary bilateral adrenal hyperplasia and was referred to endocrine surgery who recommended unilateral adrenalectomy. The decision was made to remove the larger left side adrenal mass. On post-operative day one her am cortisol decreased to 2.1 and she was started on hydrocortisone 20 mg in the morning and 10 mg in the evening. Discussion: Primary bilateral adrenal hyperplasia is a rare cause (< 2 %) of endogenous Cushing’s syndrome, usually occurs in a bimodal age distribution, in childhood and in the fifth- sixth decades. Presentation is variable with most patients having no symptoms or subclinical Cushing’s. The theory is the larger nodule size corelates with the higher cortisol production. Studies have shown between 60-70% of cases has aberrant ectopic hormone receptors which leads to increased cortisol production not only from ACTH but also from other ligands such as serotonin and vasopressin. Aberrant receptor testing examines whether cortisol or other steroid production increases in response to either physiologic or pharmacologic stimulus. Multiple genetic mutations have been associated, the most frequent is mutations in the Armadillo repeat- containing 5 gene identified in 2013. Treatment can either be medical or surgical. Medical therapy can be initiated if testing for an aberrant receptor is positive. In recent years there has been a trend towards doing unilateral adrenalectomy instead of bilateral, with initial remission of symptoms reported in about 84% of cases after unilateral adrenalectomy although there is a small risk of recurrence. Post operatively after unilateral adrenalectomy patients should be monitored for adrenal insufficiency. Our patient declined aberrant receptor testing and opted for surgery and is doing well post operatively. Conclusion: Primary bilateral adrenal hyperplasia is a rare cause of endogenous Cushing’s syndrome which can be treated either medically or surgically.

ORAL AND INTRAVENOUS SUPPRESSION TESTS IN THE DIAGNOSIS OF CUSHING'S SYNDROME

1965 ◽  
Vol 33 (3) ◽  
pp. 515-524 ◽  
Author(s):  
V. H. T. JAMES ◽  
J. LANDON ◽  
V. WYNN
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Early Period ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Adrenal Tumour ◽  
Bilateral Adrenal Hyperplasia ◽  
Steroid Excretion ◽  
Synthetic Glucocorticoids ◽  
Urinary Steroid

SUMMARY Adrenocortical suppression tests, based on the fall in urinary 17-hydroxy-corticosteroid excretion during the oral administration of dexamethasone, were found to be of value in the diagnosis of Cushing's syndrome, but less useful in differentiating bilateral adrenal hyperplasia from adrenal tumour. Such tests have the disadvantage of requiring accurate urine collections and of taking several days to perform. A test is described, based on the decrease in plasma cortisol concentration during i.v. infusion of dexamethasone at a rate of 1 mg./hr. The results obtained in 12 patients with Cushing's syndrome and bilateral adrenal hyperplasia differed from those found in control subjects in that there was a delay between the start of the infusion and the fall of plasma cortisol, and the rate of fall was less rapid. The values found after 180 min., expressed either as μg./100 ml. or as a percentage of the resting level, differed significantly (P < 0·001) in the two groups. The test proved valuable as an aid to the diagnosis of Cushing's syndrome, was easy to perform, and could be completed in 3 hr. In some patients with Cushing's syndrome, the administration of synthetic glucocorticoids appeared to result in an increased urinary steroid excretion. A transient increase in plasma cortisol levels was also observed in some of these patients during the early period of dexamethasone infusion. It is thought that this finding reflects an alteration in steroid metabolism induced by dexamethasone and fluorocortisol.

A CASE OF CUSHING'S SYNDROME STUDIED BY URINARY CORTICOTROPHIN ASSAY

1963 ◽  
Vol 42 (2) ◽  
pp. 163-167 ◽  
Author(s):  
Masami Nakamura ◽  
Masaji Hirano ◽  
Hiroshi Ibayashi ◽  
Takuo Fujita ◽  
Nakaaki Ohsawa ◽  
...  
Keyword(s):  
Diurnal Variation ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Abnormal Response ◽  
Before And After ◽  
Total Adrenalectomy ◽  
Bilateral Adrenal Hyperplasia ◽  
Distinct Increase ◽  
Daily Total

ABSTRACT A 20 year old female developed typical features of Cushing's syndrome, with increased urinary and plasma 17-OHCS, which showed a distinct diurnal variation, exaggerated response to exogenous ACTH, and suppression of response to 3 mg dexamethasone daily. Total adrenalectomy was performed for bilateral adrenal hyperplasia. ACTH in urine showed a distinct increase before and after operation. More than 30 mg of cortisol was required to suppress the elevated urinary ACTH, suggesting an abnormal response of the pituitary gland to exogenous steroid.

Bilateral adrenal hyperplasia: a rare cause of Cushing's syndrome in chidren

2014 ◽  
Author(s):  
Akshatha Taranath Kamath ◽  
Shiny Goyal Mandliya ◽  
Sanjay Rao ◽  
Sampath Satish Kumar
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Bilateral Adrenal Hyperplasia

CUSHING'S SYNDROME ASSOCIATED WITH A »CORTICOTROPHIN«-PRODUCING BRONCHIAL NEOPLASM

1967 ◽  
Vol 56 (2) ◽  
pp. 321-332 ◽  
Author(s):  
J. Landon ◽  
V. H. T. James ◽  
W. S. Peart
Keyword(s):  
Side Effects ◽  
Cushing’S Syndrome ◽  
Bronchial Carcinoma ◽  
Cushing's Syndrome ◽  
Adrenal Function ◽  
Clinical Usefulness ◽  
Adrenal Hyperplasia ◽  
Melanocyte Stimulating Hormone ◽  
Bilateral Adrenal Hyperplasia ◽  
Chemical Evidence

ABSTRACT A patient is reported who developed bilateral adrenal hyperplasia and Cushing's syndrome in association with an undifferentiated bronchial carcinoma. »Corticotrophin« was demonstrated by radioimmunoassay in the tumour and its metastases and »melanocyte-stimulating hormone« by bioassay in a metastasis. Treatment with Metyrapone resulted in bio-chemical evidence of improvement but side-effects limited its clinical usefulness. The use of pituitary-adrenal function tests in distinguishing patients with this syndrome from those with Cushing's disease is discussed.

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