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PubMed1996

[Diagnosis, surgical treatment and prognosis in endocrine hypertension of adrenal origin].

162 patients with adrenal hypertension have been examined. 48 of them had Cushing's syndrome, 10--aldosteromas, 85 --icenko--Cushing disease. The examination of patients with endocrine hypertension included hormonal background study, computer and MR tomography thin-needle biopsy and made it possible to make valid clinical and topical diagnosis in all cases. Magnetic-resonance tomography appeared to be the most efficient diagnostic method in the syndrome of hypercorticoidism. Ultrasound examination is the most available and highly informative method of revealing hormonally active adrenal tumors when they are more than 1 cm in size. An adequate preoperative preparation equally with an optimal scope of surgery and efficient postoperative substitution hormonal therapy are key factors in treatment of endocrine adrenal hypertension.

17-Hydroxycorticosteroids↗
PubMed2006

Assessing adrenal function in primary care settings with a single sample subcutaneous glucagon test.

OBJECTIVE: To test the efficacy of the low-dose glucagon test in assessing adrenal gland function. STUDY DESIGN: Subcutaneous glucagon was used to assess the hypothalamo-pituitary-adrenal gland (HPA) axis in 215 healthy children. Concordance of this test with the low-dose intravenous ACTH test was established for 42 children. Glucagon testing was conducted for 150 minutes after subcutaneous glucagon administration and for 30 minutes after 1 microg intravenous ACTH. RESULTS: Mean peak serum cortisol concentrations were 22.4 +/- 0.6 microg/dL (SEM) after subcutaneous glucagon and 20.0 +/- 0.6 microg/dL after intravenous ACTH. Specificity of 95% was found at peak cortisol concentrations of 9.5 and 12.5 microg/dL for the glucagon and ACTH tests, respectively. Concordance between the glucagon and ACTH tests was 90.5%. CONCLUSIONS: The glucagon test was found to be as good a test of the HPA axis as the ACTH test and had a 90.5% concordance with it. The ease of performing the glucagon test, namely, obtaining a single sample of blood 150 minutes after the subcutaneous administration of glucagon, makes it a useful method of assessing the HPA axis in primary care settings.

Adolescent↗
PubMedDate not supplied

[Potentialities of computed tomography and ultrasound in diagnosis of hormonally active adrenal diseases: results of comparison CT and US with operative adn histological data].

The data given in the paper suggest that X-ray computed tomography (CT) is highly effective in detecting all types of hormonally active adrenal abnormalities. CT used in hormonally active adrenal diseases yielded data on major quantitative and qualitative (primarily densitometric) criteria that could be used in assessing the images of the adrenal area in these patients. Ultrasound study (USS) made at the first stage of topical diagnostic searches was of informative value in detecting adrenal tumor lesions, the technique being highly sensitive in the diagnosis of adrenal pheochromocytomas and adenocarcinomas, but less informative in the detection of hormonally active adrenocortical adenomas (aldesterone-producing ones in particular) than CT. The diagnosis of various adrenocortical hyperplasies and the differentiation of hyperplastic and tumor forms of hypercorticoidism are a prerogative of CT that substantially supplements USS findings in such cases.

Adenocarcinoma↗
PubMed1990

New aspects of mineralocorticoid hypertension.

Primary aldosteronism is the principal disorder of the zona glomerulosa, and a number of subsets have been identified: unilateral adenoma, bilateral micro- or macronodular hyperplasia (idiopathic aldosteronism), primary hyperplasia, and aldosterone-producing carcinoma, either adrenal or ectopic. The diagnostic criteria for a correct differential diagnosis of these subsets are now quite reliable, and our experience is presented in detail. Unfortunately, the pathogenesis of most of these forms is still poorly recognized and requires further investigation. An extreme sensitivity to angiotensin II is present in patients with idiopathic aldosteronism, and a role of adrenal renin is now being advocated. A peculiar form of hyperaldosteronism is the glucocorticoid-remediable subtype. An unusual sensitivity of aldosterone to ACTH is present in this form. The qualitative biochemical abnormality in this disorder consists of a marked overproduction of products of the cortisol C-18-oxidation pathway, 18-hydroxycortisol and 18-oxocortisol, which are more abundant than aldosterone and 18-hydroxycorticosterone. A family with 3 affected sibs has been studied by our group. In other clinical situations, classical zona fasciculata mineralocorticoids (deoxycorticosterone [DOC], corticosterone, and their 18-hydroxy compounds) are secreted in excess. The hypertensive diseases of this zone are rare DOC-secreting tumors and two forms of congenital adrenal hyperplasia, the 11 beta-hydroxylase and 17 alpha-hydroxylase deficiency syndromes, which are identified by the presence of hypokalemia and suppressed renin activity. DOC is the only mineralocorticoid hormone (MCH) oversecreted in the 11-hydroxylase deficiency syndromes, while all ACTH-dependent MCH levels are very high in the 17-hydroxylase deficiency syndromes.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenoma↗
PubMedDate not supplied

Imaging features of uncommon adrenal masses with histopathologic correlation.

Uncommon adrenal masses include cystic lesions (hydatid cyst, endothelial cyst), solid lesions (hemangioma, ganglioneuroma, angiosarcoma, primary malignant melanoma), and solid fatty lesions (myelolipoma, collision tumor). Most of these lesions do not have specific imaging features. The liquid content of adrenal cysts is clearly demonstrated on ultrasonographic scans, computed tomographic scans, and magnetic resonance images. Nevertheless, the histologic type cannot be predicted except at some stages of hydatid disease in which pathognomonic features are present. The most typical imaging features of hemangioma are phleboliths and enhancement of peripheral vascular lakes. Ganglioneuroma has nonspecific radiologic features, but this diagnosis should be considered in cases with early enhancement of fine septa and progressive filling. The radiologic features of angiosarcoma and primary malignant melanoma are nonspecific. A macroscopic lipid content within an adrenal mass is theoretically characteristic of myelolipoma. This diagnosis should be made with caution, especially when the lipid content is not predominant, because of the possible association with an adenoma.

Adenoma↗
PubMed1985

The constant plasma 18-hydroxycorticosterone to aldosterone ratio: an expression of the efficacy of corticosterone methyloxidase type II activity in disorders with variable aldosterone production.

Aldosterone and 18-hydroxycorticosterone (18-OHB) are produced by the adrenocortical zona glomerulosa. Under normal conditions, plasma 18-OHB levels parallel and are influenced by the same trophic factors that regulate aldosterone production. To evaluate corticosterone-methyl-oxidase II activity, the final step of aldosterone biosynthesis, in conditions associated with chronic derangements of the pituitary-adrenal and/or renal-adrenal axis, we measured the plasma 18-OHB to aldosterone ratio, cortisol, PRA or plasma renin concentration, and potassium (K) in 104 such patients and 15 normal subjects. The 18-OHB to aldosterone ratios in the pituitary-adrenal group were not significantly different from normal regardless of elevated or reduced ACTH and/or cortisol levels [patients with Cushing's syndrome, 3.55 +/- 0.68 (+/-SE); ACTH deficiency, 2.03 +/- 0.34; 21-hydroxylase deficiency, 3.09 +/- 0.23; normal subjects, 2.50 +/- 0.15]. The renal-adrenal group also had normal ratios regardless of plasma renin concentration and K levels [patients with aldosterone-producing adenomas, 2.85 +/- 0.15; idiopathic hyperaldosteronism, 2.14 +/- 0.19; salt-losing nephropathy, 3.06 +/- 0.54; Bartter's syndrome, 2.89 +/- 0.20; isolated (hyporeninemic) hypoaldosteronism, 3.20 +/- 0.39]. Only in patients with 17 alpha-hydroxylase deficiency (230.1 +/- 118.6) was the ratio abnormally high. Chronic perturbations of aldosterone production by ACTH, the renin-angiotensin system, and/or K did not alter this last step of aldosterone biosynthesis (corticosterone-methyloxidase II), as defined by their plasma concentrations. Any influence of these trophic factors must be proximal to the site of 18-OHB production.

18-Hydroxycorticosterone↗
PubMed2003

Laparoscopic adrenalectomy for incidentaloma and bilateral adrenal disease.

Adrenalectomy is ideally suited to minimally invasive surgery based on the characteristics of adrenal tumours, which are usually small and benign. The aim of this study was to verify that laparoscopic adrenalectomy is minimally invasive and to assess the indication of laparoscopic adrenalectomy for incidentaloma. From October 1995 through August 2002, 133 patients underwent adrenal surgery at the Department of Surgery II, Nagoya University School of Medicine. Of these, 111 underwent laparoscopic adrenalectomy. All laparoscopic adrenalectomies were performed using the transabdominal lateral approach. In 50 of 133 patients, the adrenal tumours were incidentally discovered. There were 27 non-functioning adrenal tumours and six of seven preclinical Cushing's test syndrome cases incidentally discovered. Six of 27 non-functioning adrenal tumour patients underwent open adrenalectomy because of large tumour size or malignancy. Based on the present criteria for laparoscopic adrenalectomy, 15 of 133 patients were retrospectively considered to have required open adrenalectomy. The average size of a non-functioning adrenal tumour was 5.8 cm in diameter, which was equal to the average size of a phaeochromocytoma. A simultaneous bilateral laparoscopic adrenalectomy was performed in a patient in poor condition with advanced Cushing's syndrome due to adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia (AIMAH). The adrenal glands were successfully removed without fragmentation in this patient, and the postoperative course was uneventful, thanks to the minimally invasive surgery. The laparoscopic technique assures less morbidity and faster recovery, and appears to be equally effective in eradicating functioning and non-functioning adrenal masses. The benefits of the laparoscopic approach to adrenalectomy should not result in a more aggressive attitude toward the excision of clinically silent, benign-appearing adrenal incidentalomas.

Adenoma↗
PubMed1995

Adrenal lesion in multiple endocrine neoplasia type 1.

BACKGROUND: Multiple endocrine neoplasia (MEN) type 1 is accompanied by adrenal involvement, but characteristics and clinical handling of this lesion have been insufficiently explored. METHODS: Patients with MEN 1 (n = 43) were monitored (mean, 6.3 years) with annual biochemical and radiologic adrenal evaluation. Adrenal specimens were examined by in situ RNA-RNA hybridization for expression of the MEN1 candidate gene phospholipase C beta 3 (PLC beta 3) and immunostaining for insulin-like growth factor-1 receptor. RESULTS: Altogether 17 patients (40%) displayed adrenal enlargement, which was limited to the adrenal cortex and showed signs of progression, marked atypia, and cancer development in three of them. Only the carcinoma exhibited adrenocortical hormone excess. PLC beta 3 was expressed in the hyperplastic and adenomatous proliferation but not the carcinoma. Pancreatic endocrine tumors with insulin-proinsulin excess were overrepresented in the patients with adrenocortical involvement, but significant insulin-like growth factor-1 receptor immunoreactivity was restricted to the carcinoma. CONCLUSIONS: The prevalent adrenocortical lesion associated with MEN 1 requires regular attention because of malignant potential. It was unrelated to loss of constitution heterozygosity for the MEN1 locus (11q13) and PLC beta 3 expression, except for the cortical carcinoma exhibiting allelic losses involving also the Wiedemann-Beckwith gene at 11p15. Mechanisms for mitogenic relationships between the pancreatic and adrenal lesions of MEN 1 demand further clarification.

Adrenal Gland Diseases↗
PubMed1983

131I-iodocholesterol (NP-59) scintigraphy in adrenocortical diseases.

The diagnostic usefulness of adrenal imaging with 131I-iodocholesterol (NP-59) is now well established. In order to correlate histopathology with the adrenal scan the authors examine and report their experience in 37 patients with surgically proven adrenal lesions or pituitary adenomas. This series included 24 patients with Cushing's syndrome: 14 caused by benign adrenal adenoma and 10 due to bilateral pituitary-ACTH-dependent adrenocortical hyperplasia. Ten patients with primary aldosteronism were submitted to surgery which confirmed the presence of aldosterone-producing adrenal adenomas. Two women with hyperandrogenism (due to virilizing ovarian tumors) had normal adrenals but the ovarian stromal luteoma markedly concentrated the iodocholesterol while the arrhenoblastoma did not. A patient with adrenal hematoma is also included in this report. The overall histopathological correlation with the radiocholesterol scintiscan yields an accuracy in our series of 97% (36/37). The false-negative adrenal scan (also missed by other non-invasive techniques) occurred in a patient with an aldosterone-producing adrenal adenoma measuring 1.0 X 1.5 cm.

19-Iodocholesterol↗
PubMed1997

[Laparoscopic adrenalectomy].

Minimally-invasive adrenalectomy is a new and attractive procedure, which has advanced to the method of choice in the surgical treatment of benign adrenal diseases. This technique is contraindicated in the treatment of adrenal malignancies. The endoscopic removal of large benign adrenal tumors (> 6-8 cm) may be difficult. The preoperative management of the patients does not differ from the preparation for conventional adrenalectomy. Like in open adrenalectomy several different endoscopic approaches to the adrenals have been described: the transperitoneal approach with the patient in supine position, the transperitoneal approach in lateral position, the retroperitoneal approach in lateral position and the retroperitoneal approach in prone position. Excellent results have been achieved with all four techniques. The approaches with the patient in lateral position have the disadvantage that in case of bilateral adrenalectomy the patient has to be repositioned during the operation. Large tumors are more difficult to be removed with the retroperitoneal techniques, because of the limited space in the retroperitoneum. Operative times and conversion rates have been markedly reduced with increasing experience with these techniques.

Adrenal Gland Diseases↗
PubMed2004

Clinical and molecular genetic studies of bilateral adrenal hyperplasias.

ACTH-independent, cortisol-producing hyperplasia is caused by 2 distinct disorders, primary pigmented nodular adrenocortical disease (PPNAD) and ACTH-independent macronodular adrenal hyperplasia (AIMAH). We will review recent findings on the clinical and molecular aspects of PPNAD and AIMAH. Inactivating mutations of PRKAR1A on 17q22-24, which codes for the type 1A regulatory subunit of protein kinase A, have been found in a subgroup of patients with PPNAD with and without Carney complex. AIMAH is a rare condition in which cortisol secretion may be mediated by non-ACTH circulating hormones such as gastric inhibiting polypeptide (leading to food-dependent Cushing's syndrome), vasopressin, catecholamines, luteinizing hormone, serotonin, angiotensin-II or leptin. The primary etiology of AIMAH remains unclear. Recently, we studied the expression profile of AIMAH by genomic cDNA microarray analysis. Several candidate genes were identified, suggesting pathways that affect the cell cycle, adhesion and transcription as possible mediators of adrenocortical hyperplasia.

Adrenal Gland Diseases↗
PubMedDate not supplied

Computed tomographic quantification of canine adrenal gland volume and attenuation.

We conducted a retrospective study in presumed normal dogs to determine the adrenal gland attenuation and volume values. Multidetector computer tomography (MDCT 16) analysis of the gland was carried out in 48 adult dogs without evidence of adrenal gland disease that underwent CT examination for acute spinal injuries. The mean nonenhanced attenuation value +/- SD of the left adrenal gland was 36.0 +/- 5.3 HU (range: 22.0-42.0 HU). The mean nonenhanced attenuation value +/- SD of the right gland was 34.3 +/- 7.0 HU (range: 20.4-48.6HU). The mean enhanced attenuation value +/- SD were: left gland 101.5 +/- 10.6HU (range: 86.8-128.0 HU), and right gland 97.4 +/- 12.4 HU (range: 58.9-123.6 HU). The mean CT volume +/- SD were: left gland was 0.60 cm3 (range: 0.20-0.95; SD 0.17), and right gland (0.55cm3, range: 0.22-1.01; SD 0.19). Attenuation values and volume data were related to age, weight, and gender, using ANOVA. There was no statistically significant difference between the left and right side or in adrenal measurements, because of body weight class effects. The animal effect was the most important source of variation for all adrenal measurements. Based on our study, CT is an effective method for assessing adrenal characteristics in the dog. Normative CT data are provided to allow estimation of normal adrenal gland size and volume.

Adrenal Gland Diseases↗