Adrenal tumour associated with silent 21-hydroxylase deficiency in a male or with a classic form of 21-hydroxylase defect in a female?
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Adenoma↗
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A case of silent 21-hydroxylase deficiency with persistent adrenal insufficiency after removal of an adrenal incidentaloma.
A case of an adrenal incidentaloma in a 57-year-old man with silent 21-hydroxylase deficiency is reported. Abdominal computed tomography revealed a right adrenal tumour of 6cm in diameter. There was no evidence of adrenal hormone excess. However, after surgical removal of the adrenal tumour, the patient developed acute adrenal insufficiency. Adrenocortical function has remained low ever since surgery. Pathological examination of the tumour revealed a cortical adenoma. On the basis of increased plasma renin activity and serum 17 alpha-hydroxyprogesterone concentration and decreased 21-hydroxylase activity of the adenoma tissue, the patient was diagnosed as having systemic 21-hydroxylase deficiency. There are two possible mechanisms for the persistent adrenal insufficiency; first the residual left adrenal gland may have been originally hypo-functioning for some reason and, second, the left gland may have lost the ability to regenerate following prolonged suppression by the adenoma. In cases of adrenal incidentalomas with 21-hydroxylase deficiency, the indications for surgical removal should be carefully considered.
Adenoma↗
No overrepresentation of congenital adrenal hyperplasia in patients with adrenocortical tumours.
OBJECTIVE: The development and progression of sporadic adrenocortical tumours are poorly understood. In autopsy studies adrenocortical tumours are found in between 2 and 9% of the general population. In congenital adrenal hyperplasia (CAH), decreased production of cortisol leads to increased secretion of ACTH from the pituitary, resulting in hyperplasia of the adrenals. More than 95% of all cases of CAH are due to steroid 21-hydroxylase deficiency, resulting from mutations in the CYP21 gene. In subjects homozygous and heterozygous for CYP21 mutations, adrenocortical tumours have been found in a high frequency compared to the general population, suggesting that chronic ACTH stimulation may play a role in the development of this tumour form. In order to test whether mild undiagnosed CAH is a common predisposing factor, we screened 27 patients with sporadic adrenocortical tumours for CYP21 mutations. DESIGN: A retrospective study. PATIENTS: We screened 27 patients with sporadic adrenocortical tumours, representing both benign and malignant as well as hormonally active and silent lesions. MEASUREMENTS: Mutation analyses of the CYP21 gene was performed by allele-specific PCR on high molecular weight DNA. The method used detects the nine CYP21 mutations that are responsible for 95% of all disease-causing alleles in CAH. RESULTS: No mutations were detected in any of the 23 DNA samples that were prepared from leucocytes. In 4 cases where no leucocyte DNA was available, tumour tissue was analysed. In one of these tumours, two CYP21 mutations, V281 L and L307insT, were found in heterozygous form. CONCLUSION: Our data indicate that mild undiagnosed congenital adrenal hyperplasia is not a common underlying factor predisposing to adrenocortical tumours, at least not in the Swedish population.
Adrenal Cortex Neoplasms↗
New mechanisms of adrenostatic compounds in a human adrenocortical cancer cell line.
BACKGROUND: Adrenostatic compounds are frequently used in the treatment of patients with Cushing's syndrome and act via direct inhibition of steroidogenic enzymes. However, additional mechanisms may be involved in the blockade of adrenal steroid secretion. We therefore investigated the effects of aminoglutethimide (AG), metyrapone (MTP) and etomidate (ETO) in the human NCI-h295 adrenocortical carcinoma cell line. MATERIALS AND METHODS: Cells were incubated with increasing doses of the adrenostatic compounds. Steroid hormone secretion (cortisol, 17-OH-progesterone, DHEA-S) and cAMP synthesis were determined and Northern blot analysis and cell proliferation experiments were performed. RESULTS: ETO was the most potent adrenostatic compound inhibiting P450c11 hydroxylase at low concentrations (IC50 15 nM), and also blocking P450 side-chain cleavage (scc) enzyme (IC50 400 nM) at higher concentrations. The pattern of enzyme inhibition was similar for MTP with an IC50 of 3-5 microM for P450c11 and 17 microM for P450scc, while AG blocked P450scc with an IC50 of 10 microM. AG significantly suppressed the baseline ACTH-R mRNA expression in a dose-dependent fashion (300 microM AG: 5% +/- 1%; 30 microM AG: 64% +/- 1%; 3 microM AG: 108% +/- 19% compared with control cells: 100% +/- 11%) but increased glucocorticoid receptor mRNA. The reduced ACTH-R mRNA was paralleled by low ACTH-induced cAMP accumulation indicating reduced expression of ACTH-R protein. The simultaneous incubation of hydrocortisone together with AG reversed the inhibitory effect of AG on the ACTH-R expression. AG and ETO inhibited cell proliferation in the NCI-h295 cells, but ETO was much more potent and showed antiproliferative effects at concentrations of 6 microM. The growth inhibition was not reversed by administration of hydrocortisone. CONCLUSIONS: Our data demonstrate that adrenostatic compounds not only act by suppression of steroidogenic enzymes but can also influence both ACTH-R expression and cell proliferation in adrenal cells. As these effects occur in vitro at concentrations that are reached during treatment with these drugs in patients, they are probably also of clinical relevance. Particularly the antiproliferative activity of ETO may be useful in Cushing's syndrome due to adrenocortical cancer. The interaction of steroidogenesis, ACTH-R and glucocorticoid receptor expression as well as cell proliferation provides a new concept of the intra-adrenal response to stress in humans.
Adrenal Cortex Neoplasms↗
Diagnosis and treatment of adrenal tumours: a review of 35 years' experience.
OBJECTIVE: To review and analyse clinical data on the diagnosis and management of patients with adrenal masses. PATIENTS AND METHODS: Patients admitted with adrenal masses between 1960 and 1995 were reviewed. The series comprised 116 males (mean age 41.4 years, SD 10.5, range 3-77) and 95 females (mean 36.9 years, SD 11.6, range 1-62); eight patients were < 14 years old and the overall mean (SD) age was 39.4 (12.8) years. The diagnosis was based on symptoms, signs, hormone levels and imaging studies. All tumours were confirmed by surgery, and pathology and results of analysis assessed statistically. RESULTS: Over the last 35 years, the incidence of adrenal tumours was 1.7% of all patients admitted with genitourological diseases or 9.7% of patients with genitourinary tumours at our institution. The prevalence of adrenal tumours in males and females was similar but Cushing's syndrome was 3.1 times more frequent in females than in males and phaeochromocytoma 1.9 times more frequent in males than females. Most patients with adrenal tumour were aged 30-50 years. Of 211 adrenal tumours, 151 (72%) were functional, with a prevalence of benign tumour, and 60 (28%) were nonfunctional, with 35% malignant. There were 78 'incidentalomas' which included 18 functional tumours. Overall, 210 tumours were removed and one was explored. Correlation analysis between tumour size and character or hormone levels showed that size was significantly positively correlated with tumour character (r = 0.4010, P < 0.001), but there was no relationship between tumour size and hormonal levels. The postoperative complication rate was 3.3% and the mortality 0.5%. CONCLUSIONS: Based on this analysis we recommend that computed tomography is the first method used to define and localize the adrenal masses, that hormone levels should be determined in symptomatic or asymptomatic patients with adrenal masses, and that functional adrenal tumours and solid incidentalomas of any size should be removed surgically.
Adolescent↗
Unique cases of unilateral hyperaldosteronemia due to multiple adrenocortical micronodules, which can only be detected by selective adrenal venous sampling.
Primary aldosteronism is classified as aldosterone-producing adenoma (APA), idiopathic hyperaldosteronism (IHA), unilateral adrenal hyperplasia (UAH), primary adrenal hyperplasia (PAH), adrenal cancer, and glucocorticoid-remediable aldosteronism. We describe here 4 cases of primary aldosteronism due to unilateral hyperaldosteronemia, demonstrating unique histopathologic findings, such as unilateral multiple adrenocortical micronodules in the affected adrenals. Thirty-three patients with primary aldosteronism were consecutively admitted; 27 of them were treated by unilateral adrenalectomy. Four of them also had unilateral adrenal hypersecretion of aldosterone by selective adrenal venous sampling and adrenocortical multiple micronodules without an adenoma. These patients had hyporeninemic hyperaldosteronism with normokalemic hypertension. In these patients, furosemide plus upright test failed to increase plasma renin activity (PRA); the ratio of plasma aldosterone concentration (PAC) to PRA at 90 minutes after captopril administration was similar to that in patients with IHA and APA. Aldosterone concentrations were increased in each unilateral adrenal vein, and poorly encapsulated multiple adrenocortical micronodules from 2 to 3 mm in diameter were microscopically detected in the resected adrenal glands. Immunohistochemical analysis of steroidogenic enzymes, including cholesterol side chain cleavage, 3beta-hydroxysteroid dehydrogenase, 21-hydroxylase, 17alpha-hydroxylase, and 11beta-hydroxylase, indicated that the cortical cells within these micronodules were active in aldosterone production, while the non-nodular zona glomerulosa cells were inactive. We conclude that the clinical and pathologic characteristics of our 4 cases with unilateral multiple adrenocortical micronodules (UMN) are distinct from those of APA, IHA, UAH, and PAH. Furthermore, unilateral hyperaldosteronemia induced by UMN may be frequently misdiagnosed, because standard imaging tests, which cannot always detect tiny abnormalities of adrenals, showed "normal adrenal glands" in these patients. Thus, primary aldosteronism due to UMN should be carefully examined for differential diagnosis of each form of hyperaldosteronemia.
Adenoma↗
Adrenal masses in the newborn.
The incidence of neonatal adrenal tumors is increasing due to the expanded use and accuracy of prenatal ultrasonography in routine obstetric care. Although adrenal and juxtarenal masses may represent benign lesions (adrenal hemorrhage, subdiapragmatic extralobar pulmonary sequestration), the majority of masses either are premalignant or malignant. Previous algorithms for the diagnosis and management of these lesions have been guided primarily by the high incidence of neuroblastomas within this group. Improved insight into the relatively benign behavior of many neonatal neuroblastomas has stimulated debate regarding the appropriate management schema for neonatal adrenal masses. Moreover, the increasing recognition of benign juxtarenal lesions further challenges the conventional dogma. This review discusses the major categories of adrenal masses to help generate a rational algorithm for diagnosis and therapy.
Adrenal Cortex Neoplasms↗
[Supra-renal venography in patients with suspected endocrine hypertension (author's transl)].
In forty patients suspected clinically of having an endocrine cause for hypertension, an adrenal cortical adenoma was confirmed histologically in five. Hyperaldosteronism with enlarged or normal adrenals was demonstrated in three patients. The diagnosis in a further eight patients with an abnormal hormone pattern and/or an abnormal venogram has not yet been confirmed. Adrenal glands show considerable variation in their size, shape and localisation on venography.
Adenoma↗
[Cushing's syndrome. II. ACTH excess].
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Adenoma↗
[Differential diagnosis of space-occupying adrenal masses].
Computed tomography (CT) and magnetic resonance (MR) imaging are first line modalities in the evaluation of patients with adrenal gland masses, and have the potential to be very accurate for the localization of adrenal gland masses in patients with diseases associated with hyperfunctioning conditions of the adrenal gland. Both CT and MR imaging allow a specific diagnosis of acute adrenal hemorrhage, adrenal myelolipoma, and adrenal cysts. CT is also helpful in the assessment of patients with Addison's disease, particularly the subacute form secondary to granulomatous diseases. Quantitative evaluation of adrenal masses on unenhanced CT scans and/or qualitative analysis on chemical-shift MR imaging have been shown to be accurate in distinguishing adrenal adenomas from non-adenomas. Attenuation of 11 HE or less on unenhanced CT scans and/or signal loss on opposed phase MR images indicate adenoma with a high specificity and acceptable sensitivity. More recently, delayed-enhanced CT has yielded higher sensitivity and specificity values in distinguishing between adrenal adenomas and non-adenomas than both unenhanced CT and chemical-shift MR imaging do. On delayed-enhanced CT scans, adrenal adenomas exhibit a greater washout of contrast material than do adrenal non-adenomas. Therefore, adrenal non-adenomas have significantly higher attenuation than adenomas on delayed-enhanced CT scans obtained at several arbitrarily chosen time points (3-60 min) after the initiation of contrast material administration.
Adenoma↗
A detailed investigation of hirsutism in a Turkish population: idiopathic hyperandrogenemia as a perplexing issue.
Hirsutism is a common clinical problem in women and the treatment depends on the cause of hirsutism. The study was designed to determine the various causes of hirsutism and their prevalences in a Turkish population. 168 women with hirsutism attending to Endocrinology Outpatient Clinic of Erciyes University Hospital were investigated in detail. Medical history, physical examination, and basal levels of free testosterone (fT), androstenedione, follicle-stimulating hormone (FSH), luteinizing hormone (LH), dehydroepiandrosterone-sulphate (DHEAS), 17 hydroxyprogesterone (17-OHP), 11-deoxycortisol (11-S), thyroid hormones, thyroid stimulating hormone (TSH), and prolactin were determined. ACTH stimulation test was performed for the diagnosis of non-classic congenital adrenal hyperplasia (NCAH). Pelvic/vaginal and adrenal ultrasonographies were performed for the detection of tumors and polycystic ovarian changes. Polycystic ovary syndrome (PCOS) was diagnosed in 96 (57.1 %) patients, idiopathic hirsutism in 27 (16 %), NCAH in 12 (7.1 %), adrenal carcinoma in 3 (1.8 %), and Cushing's disease in 1 (0.6 %) patient. Among patients with NCAH, 11 (91.7 %) patients had 11-beta hydroxylase (11-beta OH) deficiency, and 1 (8.3 %) had 21-hydroxylase deficiency. The etiology of hyperandrogenemia was not clear in 29 (17.4 %) patients and these patients were named as idiopathic hyperandrogenemia. The clinical presentation of 11-beta OH deficiency is indistinguishable from that of other hyperandrogenic states and ACTH stimulation test is the only way to diagnose this entity. Although PCOS is the most common cause of hirsutism, it is notable that nearly one fifth of hirsute women have no apparent cause of hyperandrogenemia.
Adrenal Cortex Neoplasms↗
[The value of fine needle puncture in adrenal gland tumors].
UNLABELLED: The value of fine needle biopsy (FNB) in adrenal masses was investigated in 33 patients presenting with such masses. Representative material was obtained 18 times from 19 patients with a history of tumour. 4 of them were diagnosed to be genuinely benign and 14 to be genuinely malignant. Further representative material was obtained 13 times from 14 patients with incidental findings of adrenal masses. The material gained by puncturing was classified correctly as benign in 9 cases, and twice it was considered benign although the tumours later proved malignant. In one case a clinically not suspected malignant lesion was detected by FNB, while in another case a malignant lesion was suspected by FNB, whereas a benign tumour was proven by means of surgery. The share of malignant diagnoses corresponded with the size of tumour, ascertained by the application of ultrasound or CT. The rate of malignant adrenal masses, which were found incidentally, increased from 7 cm onwards; however, the rate of malignant adrenal masses obtained from patients with a history of tumour increased distinctively already from 4 cm onwards. From 11 patients out of 33, adrenal tissue was obtained and classified as benign lesion (adenoma), although, by means of FNB and the conditions for reasons of method, a well differentiated carcinoma cannot be excluded. CONCLUSION: FNB is indicated for non-functioning tumours examined in patients with a history of tumour, furthermore, for incidental findings of the size between 4-6 cm. A diagnostic approach to adrenal masses is suggested.
Adrenal Cortex Neoplasms↗
Evidence of 11 beta-hydroxylase deficiency in a patient with cortical adrenal adenoma.
We explored a 61 year old woman with mild hirsutism. An adrenal tumor was found in the left adrenal, which was held responsible for the androgen secretion. The in vitro incubation of the tumor tissue showed an impaired 11 beta-hydroxylation of 11-deoxycortisol. This is a rare and unusual case of adrenal pathology showing that a deficiency in 11 beta-hydroxylase activity does not rule out the presence of an adrenocortical adenoma.
Adenoma↗
Lack of inhibitory effect of alpha-human atrial natriuretic polypeptide on cortisol secretion in cultured adrenocortical adenoma cells from the patients with Cushing's syndrome.
The effects of synthetic alpha-human atrial natriuretic polypeptide (alpha-hANP) on cortisol secretion by adrenocortical adenoma cells from patients with Cushing's syndrome (CS cells) in primary monolayer cultures, compared to cultured normal adrenal cells, were studied. alpha-hANP significantly inhibited cortisol secretion by human normal adrenal cells in culture, but had no direct effect on cortisol secretion from CS cells, in the presence or absence of 10(-8) M ACTH. alpha-hANP enhanced the accumulation of intracellular cyclic GMP in normal adrenal cells in culture, but not in CS cells. Visualization of [125I] iodo-alpha-hANP-specific binding sites by an in vitro receptor autoradiographic technique showed that these sites were lacking in adrenocortical adenoma tissues. These results suggest that the loss of alpha-hANP inhibitory effect on cortisol secretion in CS cells may be due to the absence of alpha-hANP receptor sites.
Adenoma↗
[Diagnosis of adrenal cortex diseases].
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Adrenal Cortex Diseases↗
[Therapy of adrenal cortex diseases].
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Acute Disease↗
[Female pseudohermaphroditism in congenital adrenogenital syndrome as an incidental intraoperative finding in a 68 year old patient].
HISTORY AND CLINICAL FINDINGS: A 68-year-old man of small stature, previously always healthy and with a grown-up daughter, was suspected of having carcinoma of the colon with metastasis to the right kidney. At laparotomy internal female genitalia with cancerous changes were unexpectedly discovered in the left adnexae. The colon carcinoma, the right adrenocortical tumour and left adnexae were resected. INVESTIGATIONS: Histological examination revealed adenocarcinoma of the colon, right adrenocortical adenoma and a Brenner tumour of the left female adnexae. Postoperative tests showed increased levels of 17-OH-progesterone (3192 ng/dl), 21-desoxycortisol (1856 ng/dl) and of adrostenedione (745 ng/dl), while the concentrations of 17-OH pregnenolone, testosterone and mineralocorticoids were within normal limits. Chromosome analysis demonstrated karyotype 46 XX. DIAGNOSIS, TREATMENT AND COURSE: As far as could be ascertained, this is the first documented case in the German-speaking region of female pseudohermaphroditism diagnosed in an elderly person with uncomplicated virilizing congenital adrenogrenital syndrome due to 21-hydroxylase deficiency (deletion of CYP21 gene). To avoid a cortisone deficiency crisis the patient was regularly given hydrocortisone and he quickly recovered. But he died 6 months later of sequelae of the carcinoma of the colon. CONCLUSION: An adrenogenital syndrome should be excluded in a case of bilateral adrenocortical tumour. As this is usually benign, conservative treatment should be attempted. This case demonstrates the necessity of thorough examination which could have given an early indication of the underlying condition.
Adrenal Cortex Neoplasms↗
Gene expression and roles of angiotensin II type 1 and type 2 receptors in human adrenals.
Although stimulation of aldosterone secretion is one of the functions of angiotensin II, the gene expression and biological significance of the angiotensin II receptor subtypes, AT1 and AT2, in the human adrenal have not been characterized. We therefore investigated the transcription levels of the receptor subtype genes and their roles in regulation of steroid secretion by human adrenals. The expression of AT1 and AT2 receptor mRNA was assessed by reverse transcription-polymerase chain reaction followed by Southern blot analysis in normal adrenocortical tissues (n = 6) and a series of adrenal tumour tissues: aldosterone-producing adrenocortical adenoma (n=6), Cushing's syndrome (n = 6) and pheochromocytoma (n = 6). The role of the two receptor subtypes in steroid secretion in vitro was examined by incubating the tissue with angiotensin II(1 microM) with or without the selective AT1 antagonist CV-11974 (1 microM). Both AT1 and AT2 receptor mRNA transcripts were demonstrated in all of the human adrenal tissues tested. Angiotensin II-induced aldosterone secretion was suppressed 50% upon the addition of CV-11974. The selective AT2 agonist CGP-42112 increased aldosterone secretion by 55% over the control, which was not suppressed by CV-11974. Angiotensin II and CGP-42112 did not affect cortisol secretion. These results suggest that both AT2 and AT1 receptors may be involved in the regulation of aldosterone secretion and tumorigenesis of the human adrenals.
Adenoma↗