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

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

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Adrenocortical adenoma in a six-year-old Egyptian boy

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1043 ◽

2021 ◽

Vol 2 (2) ◽

Author(s):

Eman B Kamaleldeen ◽

◽

Shimaa Kamal Mohamed ◽

Kotb Abbass Metwalley ◽

Hamdy M Ibrahim ◽

...

Keyword(s):

Cushing’S Syndrome ◽

Cushing Syndrome ◽

Histopathological Examination ◽

Adrenal Adenoma ◽

Time Lag ◽

Laboratory Data ◽

Clinical Signs ◽

Cushing's Syndrome ◽

Additional Treatment ◽

Adrenocortical Adenoma

Although adrenal cortical tumors are rare in children, pediatricians should be very cautious about it. Neoplasm is a rare but significant cause of Cushing’s syndrome. Unfortunately, they can be missed very easily, as there is often a time lag between the onset of the symptoms and final diagnosis. Here, we present a case of adrenal adenoma in a six-year - old Egyptian boy who regrettably misdiagnosed twice as exogenous Cushing syndrome and simple obesity. Diagnosis of an adrenal cortical tumor was confirmed by laboratory data, MRI and histopathological examination after more than 2 years of the onset of his symptoms. After resection of the tumor, serum steroids normalized, and clinical signs receded. The child received no additional treatment and remains disease-free after 18 months of close observation. The possibility of neoplasms should always be considered early to avoid delayed diagnosis and treatment of Cushing’s syndrome.

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Frequency of Osteoporosis in Iatrogenic Cushing’s syndrome: Scenario of outpatient department in urban hospitals

Bangladesh Journal of Medicine ◽

10.3329/bjm.v31i1.44857 ◽

2020 ◽

Vol 31 (1) ◽

pp. 18-21

Author(s):

Tamanna Bahar ◽

Shaila Rahman ◽

Lilian Catherene Gomes ◽

Mohammad Murad Hossain ◽

Zulfia Zinat Chowdhury ◽

...

Keyword(s):

Cushing’S Syndrome ◽

Cushing Syndrome ◽

Reference Population ◽

Final Diagnosis ◽

Cushing's Syndrome ◽

Outpatient Department ◽

Clinical Feature ◽

Cross Sectional ◽

Prevalence Of Osteoporosis ◽

Iatrogenic Cushing’S Syndrome

Background: Cushing’s syndrome is caused by excessive activation of glucocorticoid receptor. Iatrogenic Cushing syndrome is the prevalent one world -wide .Patients with Cushing’s syndrome has a high prevalence of osteoporosis. Objective: To see the prevalence of osteoporosis in Iatrogenic Cushing’s syndrome patient. Method: This descriptive cross-sectional study included 211 diagnosed case of Cushing’s syndrome during the time period of December 2013 to December 2018 in outpatient department of Dhaka Medical College and Health and Hope Hospital. Final diagnosis was done on the basis of clinical feature, serum basal cortical level and BMD. We found male were prevalent one (56%).80.56% showed biochemical evidence of Cushing’s syndrome. BMD was done in 113 patients compared with a reference population by means of T score, 17.69% patient in osteoporotic range. Conclusion: The prevalence of osteoporosis and osteopenia is age and sex independent. Judicial use of steroid and co administration with calcium, bisphosphonate can prevent osteoporosis. Treatment with bisphosphonates should be considered in all patients (irrespective of age) with Cushing’s syndrome with a low BMD to reduce fracture. Bangladesh J Medicine Jan 2020; 31(1) : 18-21

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Cushing syndrome secondary to late onset adrenal hyperplasia: presentation and challenges of management

Pyramid Journal of Medicine ◽

10.4081/pjm.2018.8 ◽

2018 ◽

Vol 1 (1) ◽

Author(s):

Muzzammil Abdullahi ◽

Mamuda Atiku ◽

Imam Mohammed Ibrahim

Keyword(s):

Cushing’S Syndrome ◽

Cushing Syndrome ◽

Late Onset ◽

Adrenal Adenoma ◽

Protein Structures ◽

Cushing's Syndrome ◽

Response To Treatment ◽

Adrenal Tumors ◽

Adrenal Hyperplasia ◽

Supraclavicular Fossa

Cushing’s syndrome is a clinical disorder caused by overproduction of cortisol. Adrenal adenoma is the cause in 5% of cases of Cushing syndrome. ACTH-independent Cushing’s syndrome in 90% is caused by unilateral adrenal tumors. Of these, adenomas are the cause in 80% of the cases, while the others are adrenocortical carcinoma. Rare causes of Cushing’s syndrome include adrenal hyperplasia. Overproduction of cortisol results in weakened protein structures leading to protuberant abdomen and poor wound healing, glucose is converted to fat and deposited in the abdomen, supraclavicular fossa and cheeks. Other presentations are diabetes, hypertension, osteoporosis, fractures, impaired immune function, glucose intolerance, and psychosis. We report a 21-year-old female who presented with clinical symptoms of newly diagnosed hypertension and diabetes mellitus which after poor response to treatment she was evaluated and diagnosed to have Cushing syndrome. She subsequently had left adrenalectomy and did very well with resolution of patients’ symptoms. The histology came out to be diffuse adrenal hyperplasia.

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

Journal of Endocrinology ◽

10.1677/joe.0.0230119 ◽

1961 ◽

Vol 23 (2) ◽

pp. 119-127 ◽

Cited By ~ 9

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.

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Adrenal Cushing’s syndrome during pregnancy

Acta Endocrinologica ◽

10.1530/eje-17-0263 ◽

2017 ◽

Vol 177 (5) ◽

pp. K13-K20 ◽

Cited By ~ 12

Author(s):

C E Andreescu ◽

R A Alwani ◽

J Hofland ◽

L H J Looijenga ◽

W W de Herder ◽

...

Keyword(s):

Luteinizing Hormone ◽

Cushing’S Syndrome ◽

Adrenal Tumor ◽

Cushing Syndrome ◽

Adrenal Adenoma ◽

Cushing's Syndrome ◽

Receptor Expression ◽

Aberrant Expression ◽

Cortisol Responses

Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. CS during pregnancy is most frequently due to an adrenal adenoma and to a lesser degree to adrenocorticotropic hormone (ACTH) hypersecretion by a pituitary adenoma. Furthermore, aberrant expression of luteinizing hormone (LH) receptors in the adrenal cortex has been suggested to be involved in the pathogenesis of adrenal CS during pregnancy. We report three pregnant women with ACTH-independent Cushing’s syndrome and an adrenal tumor. After uncomplicated delivery, patient 1 underwent in vivo testing for aberrant hormone receptor expression by the adenoma. Cortisol responses were found after administration of luteinizing hormone-releasing hormone (LHRH), human chorionic gonadotropin (hCG), glucagon, vasopressin and a standard mixed meal. All patients were treated with laparoscopic adrenalectomy. Adrenal tumor tissue of two patients showed positive immunohistochemical staining of LH receptors. Considering the cortisol responses to LHRH and hCG, and the development of CS during pregnancy in these patients, it is likely that ACTH-independent hypercortisolism was induced by the pregnancy-associated rise in hCG levels that activated aberrantly expressed LH receptors in the adrenal adenoma. Remarkably, adrenal adenomas may simultaneously express multiple aberrant receptors and individual ligands may play a role in the regulation of cortisol production in CS during pregnancy.

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Adrenocorticotropic hormone (ACTH) independent Cushing's syndrome due to unilateral adrenocortical hyperplasia: two case reports

Pan African Medical Journal ◽

10.11604/pamj.2021.38.367.26040 ◽

2021 ◽

Vol 38 ◽

Author(s):

Yousra Aouinati ◽

Amal Mjabber ◽

Nassim Essabah Haraj ◽

Siham El Aziz ◽

Asmaa Chadli

Keyword(s):

Cushing’S Syndrome ◽

Adrenocorticotropic Hormone ◽

Case Reports ◽

Cushing's Syndrome ◽

Adrenocortical Hyperplasia

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

Acta Endocrinologica ◽

10.1530/acta.0.1100302 ◽

1985 ◽

Vol 110 (3) ◽

pp. 302-307 ◽

Cited By ~ 9

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.

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Clinical Characteristics for the Improvement of Cushing's Syndrome Complicated With Cardiomyopathy After Treatment With a Literature Review

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.777964 ◽

2021 ◽

Vol 8 ◽

Author(s):

Sisi Miao ◽

Lin Lu ◽

Ling Li ◽

Yining Wang ◽

Zhaolin Lu ◽

...

Keyword(s):

Cushing’S Syndrome ◽

Clinical Characteristics ◽

Adrenocorticotropic Hormone ◽

Case Reports ◽

Adrenal Adenoma ◽

Clinical Manifestations ◽

Cushing's Syndrome ◽

Imaging Results ◽

Free Cortisol ◽

Significant Difference

Background: Endogenous Cushing's syndrome (CS), also called hypercortisolism, leads to a significant increase in mortality due to excessive cortisol production, which is mainly due to cardiovascular disease. CS complicated with cardiomyopathies, which is a rare and severe condition, has rarely been reported in the literature.Objective: To investigate the clinical characteristics of CS complicated with cardiomyopathies, we retrospectively reviewed the clinical manifestations, laboratory results, cardiac imaging results and prognosis to further understand the diagnosis, treatment, and management of these cases.Methods: The clinical data of patients diagnosed with CS complicated with cardiomyopathies obtained from discharge sheets from Peking Union Medical College Hospital from January 1986 to August 2021 were collected. Case reports of CS complicated with cardiomyopathies were retrieved from PubMed. In addition, Cushing's disease (CD) patients without cardiomyopathies were collected as controls to compare the clinical features.Results: A total of 19 cases of CS complicated with cardiomyopathies and cases of CD without cardiomyopathies (n = 242) were collected. The causes of CS included pituitary adenoma (n = 8, 42.11%), adrenal adenoma (n = 7, 36.84%), ectopic adrenocorticotropic hormone (ACTH) tumor (n = 2, 10.53%) and unclear causes (n = 2, 10.53%) in the CS complicated with cardiomyopathies group. The types of cardiomyopathies were dilated cardiomyopathies (n = 15, 78.94%) and hypertrophic cardiomyopathies (n = 4, 21.05%). The serum sodium concentration was significantly higher [145.50 (140.50–148.00) mmol/L vs. 141.00 (140.00–143.00) mmol/L], while the serum potassium concentration was significantly lower [2.70 (2.40–3.60) mmol/L] vs. 3.90 (3.50–4.20 mmol/L)] in the CS complicated with cardiomyopathies group compared to the CD patients without cardiomyopathies. There were no significant differences between the CS complicated with cardiomyopathies group and the CD patients without cardiomyopathies in the serum cortisol concentration and 24-h urine free cortisol, but a significant difference in the adrenocorticotropic hormone level [109.00 (91.78–170.30) pg/ml vs. 68.60 (47.85–110.00) pg/ml]. Twelve/16 (75.0%) patients showed significant improvement or even a complete healing of the heart structure and function after remission of hypercortisolemia after treatment with CS.Conclusions: CS complicated with cardiomyopathies is a very rare clinical entity, in which cortisol plays an important role and it can be greatly improved after remission of hypercortisolemia.

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