Oestrogen and cortisol producing adrenal tumour.

Ghazi, Ali A.; Mofid, Djafar; Rahimi, Farzaneh; Marandi, Hasan; Nasri, Hamid; Afghah, Susan

doi:10.1136/adc.71.4.358

Cited by 11 publications

(11 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Two of the patients have been reported earlier (8,9) and an update on their follow-up is also included in this presentation. Three patients, numbers 1, 2 and 6, had been diagnosed as congenital adrenal hyperplasia (CAH) before being referred to our center and were under treatment with glucocorticoids.…”

Section: Methodsmentioning

confidence: 99%

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Ghazi¹,

Mofid²,

Salehian³

et al. 2013

Jcrpe

View full text Add to dashboard Cite

Objective: Adrenocortical tumors are rare childhood neoplasms. More than 95% are functional and present with virilization, Cushing’s syndrome, hypertension, or hyperestrogenism. The objective of this paper is to present the clinical, laboratory and pathological findings of this rare disease and to highlight the secretory behavior of these tumors.Methods: Clinical and laboratory data of seven Iranian children and adolescents aged between 2 and 16 years with functioning adrenocortical tumors are presented. Five patients had virilization and two had Cushing’s syndrome at the time of diagnosis. In all subjects, the tumors were removed successfully by open surgery, during which a blood sample was drawn from the corresponding adrenal vein for hormonal evaluation.Results: Peripheral blood evaluation revealed that in addition to the dominant hormone (testosterone in the cases presenting with virilization and cortisol in those with Cushing’s syndrome), significant amounts of other hormones were secreted from these tumors. Adrenal vein evaluation revealed that testosterone, dehydroepiandrosterone sulfate, estradiol, ?17(OH) progesterone, and cortisol were directly released from the tumor. The tumors weighed between 36-103 grams. The patients have since been followed for 5 to 20 years, and there have been no signs or symptoms of relapse in any of the patients.Conclusions: The study shows that functioning adrenocortical tumors should be considered in children and adolescents presenting with hyperandrogenism, Cushing’s syndrome, or hyperestrogenism. A diagnosis of a functioning adrenocortical tumor requires surgical removal as early as possible to prevent the untoward effects of virilization or corticosteroid excess. Evaluation of adrenal vein hormones showed that the steroids are secreted directly from the tumor and peripheral conversion has little contribution to the serum levels.Conflict of interest:None declared.

show abstract

Section: Methodsmentioning

confidence: 99%

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Ghazi¹,

Mofid²,

Salehian³

et al. 2013

Jcrpe

View full text Add to dashboard Cite

show abstract

“…For children, boys consulted for gynecomastia, but rarely for Cushing syndrome complications’ such as delirium and aggressive behavior as it was observed in one case. [ 31 ] In girls, the main complaint was early breast development ± menstruations. In one case, it was a sub occlusive syndrome.…”

Section: Ircumstances Of D mentioning

confidence: 99%

“…In literature only single cases have been reported in adults[ 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 19 21 22 23 24 25 25 ] and in pediatric population. [ 19 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 ] The largest published series does not exceed two[ 7 25 ] and three cases. [ 10 ] In our department we recorded five cases but only four have been published.…”

Section: Introductionmentioning

confidence: 99%

Feminizing adrenocortical tumors: Literature review

Chentli

Bekkaye

Azzoug

2015

Indian J Endocr Metab

View full text Add to dashboard Cite

Feminizing adrenal tumors (FAT) are extremely rare tumors prevailing in males. Clinical manifestations are gynecomastia and/or other hypogonadism features in adults. They are rarer in pediatric population and their main manifestation is peripheral sexual precocity.In women genital bleeding, uterus hypertrophy, high blood pressure and/or abdomen mass may be the only manifestations.On the biological point, estrogen overproduction with or without increase in other adrenal hormones are the main abnormalities. Radiological examination usually shows the tumor, describes its limits and its eventual metastases. Adrenal and endocrine origins are confirmed by biochemical assessments and histology, but that one is unable to distinguish between benign and malignant tumors, except if metastases are already present. Immunostaining using anti-aromatase antibodies is the only tool that distinguishes FAT from other adrenocortical tumors. Abdominal surgery is the best and the first line treatment. For large tumors (≥10 cm), an open access is preferred to coeliosurgery, but for the small ones, or when the surgeon is experienced, endoscopic surgery seems to give excellent results. Surgery can be preceded by adrenolytic agents such as ortho paraprime dichloro diphenyl dichloroethane (Mitotane), ketoconazole or by aromatase inhibitors, but till now there is not any controlled study to compare the benefit of different drugs. New anti-estrogens can be used too, but their results need to be confirmed in malignant tumors resistant to classical chemotherapy and to conventional radiotherapy. Targeted therapy can be used too, as in other adrenocortical tumors, but the results need to be confirmed.

show abstract

“…There are several case reports of adrenal hyperplasia, adenoma, or cancer associated with Cushing's syndrome, gynecomastia, and hypertension 9‐13 . Often, estrogen levels are increased through the peripheral aromatization of the increased secretion of adrenal androgens or excessive estrogen production.…”

Section: Secondary Causes Of Hypertensionmentioning

confidence: 99%

“…There are several case reports of adrenal hyperplasia, adenoma, or cancer associated with Cushing's syndrome, gynecomastia, and hypertension. [9][10][11][12][13] Often, estrogen levels are increased through the peripheral aromatization of the increased secretion of adrenal androgens or excessive estrogen production. Chronic, excess alcohol use, amphetamines, cyclosporine, and anabolic steroids are associated with an increase in blood pressure and gynecomastia.…”

Section: Secondary Causes Of Hypertensionmentioning

confidence: 99%

Gynecomastia and Hypertension

Prisant

Chin

2005

J of Clinical Hypertension

View full text Add to dashboard Cite

Hypertensive heart disease encompasses anatomical changes and altered physiology of heart muscle, coronary arteries, and great vessels. Left ventricular hypertrophy is not only a target organ response to increased afterload, but is also the most potent cardiovascular risk factor. Regression of hypertrophy reduces morbidity and mortality. Heart failure may be present in the absence of a reduction of myocardial contractility. Ischemic heart disease occurs in the absence of epicardial coronary disease. Left atrial size and atrial fibrillation are associated. Potentially lethal ventricular arrhythmias and sudden cardiac death are more common in hypertensive patients. The relationship of aortic root size to blood pressure is weaker than expected; however, the relationship to aortic dissection is stronger. Careful attention and treatment of left ventricular hypertrophy, heart failure, ischemic heart disease, and atrial fibrillation will improve survival.

show abstract

Oestrogen and cortisol producing adrenal tumour.

Cited by 11 publications

References 3 publications

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Feminizing adrenocortical tumors: Literature review

Gynecomastia and Hypertension

Contact Info

Product

Resources

About

Oestrogen and cortisol producing adrenal tumour.

Cited by 11 publications

References 3 publications

Functioning Adrenocortical Tumors in Children-Secretory Behavior

Functioning Adrenocortical Tumors in Children-Secretory Behavior

Feminizing adrenocortical tumors: Literature review

Gynecomastia and Hypertension

Contact Info

Product

Resources

About

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Functioning Adrenocortical Tumors in  Children-Secretory Behavior