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PubMed2001

[Laparoscopic adrenalectomy with lateral approach--a comparison with the conventional dorsal technique].

OBJECTIVES: Minimal invasive techniques are gaining more and more acceptance in adrenal gland surgery. In a matched case control study laparoscopic transabdominal adrenalectomy in the lateral position (LA) was compared to the conventional open dorsal technique (DA) with resection of the 11th or 12th rib. METHODS: Between July 1998 and March 2000, 26 LA in 24 patients (two bilateral) were prospectively documented and compared to 25 DA in 23 matched patients (two bilateral), who had been operated on between January 1995 and June 1998. Indications for adrenalectomy in all patients were benign adrenal lesions < 6 cm. RESULTS: Age, gender, average tumor size (3.5 cm/3.6 cm) and tumor types (Conn adenoma: 10/7; Cushing: 8/7, including 2 bilateral in each group; pheochromocytoma: 3/6, incidentaloma: 2/2; others: 3/3) were distributed in both groups (LA/DA) without statistical differences. However, statistically significant differences (P < 0.05) were present (LA vs DA) comparing intraoperative blood loss (200 vs 360 ml), postoperative narcotic equivalents (1.1 vs 6.2), morbidity (8 vs 30%), and length of hospital stay (5.5 vs 9 days). Average operating time was significantly longer in LA (130 vs 105 min), but decreased during the last LA cases to the DA level. One LA had to be converted to open surgery due to diffuse bleeding. Following LA we observed two minor complications (small retroperitoneal hematoma, nerve irritation below the 12th rib); following DA there were 6 minor (2 dorsal subcutaneous hematomas, 2 nerve irritations, dystelectasis, pleural effusion) and one major complication (wound infection). CONCLUSION: LA represents a safe procedure with all the common advantages of minimal access surgery. Based on our experience and that of others, laparoscopic adrenalectomy has become the gold standard for adrenalectomy in most cases of benign adrenal disease. As adrenal surgery is rare, at present LA should be restricted to centers with a special interest in endocrine and laparoscopic surgery.

Adrenal Gland Diseases↗
PubMed1996

Hormonal responses during various phases of autoimmune adrenal failure: no evidence for 21-hydroxylase enzyme activity inhibition in vivo.

Adrenal autoantibodies (ACA) are markers of adrenal cortex involvement in idiopathic Addison's disease. Recently the 21-hydroxylase (21-OH) enzyme has been discovered to be the major autoantigen of the ACA. A potential role of these antibodies in determining adrenal failure by inhibition of the 21-OH has been recently postulated. To test this hypothesis, cortisol and aldosterone (final products of adrenal steroid synthesis) and 17-hydroxyprogesterone (17-OH-progesterone) (as a marker of 21-OH impairment) have been investigated in baseline conditions and after ACTH (1-24) stimulation test in a group of 42 patients positive for both ACA and 21-OH autoantibodies. Patients were divided into five groups according to the stages (0-4) of adrenal failure. With progression toward overt Addison's disease, baseline 17-OH-progesterone, cortisol, and aldosterone remained almost unchanged but with impairment of their responses to ACTH (1-24) stimulation. The 17-OH-progesterone/cortisol ration remained normal both in basal conditions and after stimulation at stages 0-3. At stage 4 (overt Addison's disease), this ratio increased in baseline condition with no changes after ACTH (1-24), probably because of persistent 17-OH-progesterone gonadal production. In conclusion, there was a progressive and concomitant impairment of the synthesis of all steroids tested over various phases of adrenal failure. The pattern of response of the 17-OH-progesterone/cortisol ratio to ACTH stimulation in patients with 21-OH autoantibodies was not consistent with the autoantibodies inhibiting the 21-OH activity. This suggests that the inhibiting effect of 21-OH autoantibodies on 21-OH activity is not usually evident in vivo.

Adrenal Gland Diseases↗
PubMed1994

Comparison between the suppressive effects of dexamethasone and loperamide on cortisol and ACTH secretion in some pathological conditions.

Since in patients with Cushing's disease, unlike in normal subjects, tonic inhibitory opioid control of ACTH secretion does not operate, use of the opiate agonist loperamide (LOP) has recently been proposed in the diagnosis of hypercortisolemic states. We compared the sensitivity, specificity and diagnostic accuracy of the LOP test (16 mg orally) with corresponding results of the dexamethasone test (DXM, 1 mg orally overnight) in 23 normal subjects and in a total of 42 patients, affected by Cushing's disease (n = 8), incidentally discovered adrenal masses with impaired function of the hypothalamic-pituitary-adrenal (HPA) axis (n = 6), obesity (n = 21) and depression (n = 7). While in controls both DXM and LOP strongly suppressed plasma cortisol and ACTH, in Cushing's disease and in incidentalomas no patient showed a decrease in cortisol levels below 50 ng/ml or a reduction in plasma cortisol greater than 50% of basal values in response to LOP and DXM. In obese subjects both drugs significantly reduced plasma cortisol and ACTH without giving false positive results. In the depressed group only 3/7 patients showed a decrement in cortisol levels below 50 ng/ml after LOP in contrast to 6/7 after DXM. Thus, in patients with impairment of the HPA-axis, i.e. in Cushing's disease and in patients with adrenal incidentalomas and hormonal abnormalities, LOP and DXM test sensitivity was 100%. In controls and in obese patients specificity was 100% both with LOP and DXM, while in depressed patients it was 43% and 86% with LOP and DXM, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1978

Adrenal scanning and uptake with 131I-6beta-iodomethyl-nor-cholesterol.

Adrenal scans were obtained in 50 patients with suspected adrenal disease; 131I-19-iodocholesterol was used in 12 and 131I-6-beta-iodomethyl cholesterol (NP-59) in 38 cases (42 scans). The scan diagnosis was correct in 48 patients. Successful images were obtained with 0.5 mCi, or less, of NP-59 instead of the standard dose of 2 mCi, thus reducing radiation dose to the patient and cost of the agent. Satisfactory scans were obtained in 2--3 days in patients with hyperfunctioning adrenals, and in 3--7 days in other groups of patients, when NP-59 was used. With 131I-19-iodocholesterol, however, 5--10 days were required for optimal results in all patients including those with Cushing disease. Adrenal uptake studies were very useful in the evaluation of adrenal function, particularly in differentiating bilateral hyperplasia from normal adrenals.

Adrenal Gland Diseases↗
PubMed2002

The role of SF1 in adrenal and reproductive function: insight from naturally occurring mutations in humans.

Steroidogenic factor 1 is a monomeric orphan nuclear receptor and one of several hundreds of transcription factors encoded in the human genome. It regulates the transcription of many genes involved in gonadal development, sexual differentiation, steroidogenesis and reproduction. Recently, mutations in the gene encoding SF1 have been identified in several patients with primary adrenal failure and 46,XY sex-reversal. Interpreting the consequences of these mutations provides further understanding of transcription factor haploinsufficiency in human genetic disease as well as the exquisite sensitivity of humans to gene-dosage effects during adrenal and gonadal development.

Adrenal Gland Diseases↗
PubMed2004

Cystic adrenal neoplasms.

BACKGROUND: Cystic lesions of the adrenal gland are uncommon and demonstrate a spectrum of histologic changes and may vary from pseudocysts to malignant cystic neoplasms. METHODS: The authors analyzed a series of primary cystic lesions of the adrenal gland to evaluate the clinicopathologic features and histologic spectrum and to determine features that are helpful in distinguishing benign from malignant cystic adrenal tumors. RESULTS: A total of 41 cases of macroscopically cystic lesions among patients who underwent surgery at the Mayo Clinic were identified over a 25-year period. Of these 41 cases, 32 were pseudocysts, 8 were endothelial cysts, and 1 was an epithelial cyst. Of the 32 pseudocysts, 6 were associated with adrenal neoplasms, including 2 adrenal cortical carcinomas, 2 adrenal cortical adenomas, and 2 pheochromocytomas. One pheochromocytoma case was identified in association with an endothelial cyst. Both patients with cystic adrenal cortical carcinomas died of disease. CONCLUSIONS: Adrenal neoplasms, including adrenal cortical carcinomas, may be associated with benign-appearing cysts. Extensive pathologic sampling of resected tissues is important to rule out malignancy in patients with cystic adrenal lesions.

Adolescent↗
PubMedDate not supplied

Fine needle aspiration cytology of the adrenal gland.

The cytologic appearance of fine needle aspiration (FNA) specimens emanating from both symptomatic and incidental masses of the adrenal glands in 22 patients who attended a large cancer institution from 1976 to 1981 is described. Eligibility for the study required thorough clinical and radiologic follow-up for at least two years following the initial cytologic diagnosis. Histologic and ultrastructural correlations were performed when possible. Nine patients were found to have benign lesions, including five adrenal cysts, two adenomas, one nodular hyperplasia and one adrenal myelolipoma. Thirteen patients had malignant lesions, of which six were primary adrenal tumors, either neuroblastoma (two) or adrenocortical carcinoma (four). The overall sensitivity of FNA in detecting the presence of malignancy was 85%, while the number of patients correctly classified for all adrenal masses was 90%. The test was 100% specific for malignant lesions. It is concluded that FNA of adrenal masses is a safe and simple procedure with a high degree of accuracy. Its use appears to be especially justified in those patients with primary neoplasms of nonadrenal sites, in whom silent adrenal lesions are detected during radiologic surveys for metastatic disease. Fifteen of the patients fell into this category, yet over half (53%) of them were shown to have benign adrenal lesions, treatable with a conservative approach.

Adrenal Gland Diseases↗
PubMed1981

Prenatal diagnosis of congenital adrenal hyperplasia (21-OH deficiency type) by HLA typing.

The close genetic linkage between HLA-B and congential adrenal hyperplasia due to 21-hydroxylase deficiency permits prenatal diagnosis of an affected fetus by HLA typing of amniotic fluid cells in pregnancies at risk. Some families at risk, especially those with an affected girl with ambiguous genitalia, will only plan another pregnancy if a prenatal diagnosis is possible. After HLA typing of the index case, parents and eventually grandparents, the family were informed of the possibility of a prenatal diagnosis. Fibroblast cell lines were initiated from skin biopsies of the index cases and parents and were used as controls in the tests. HLA typing of the fetus was done on amniotic fluid cells grown in vitro using first, a microcytotoxicity test and second quantitative microabsorption test. Ten prenatal diagnoses are reported. In two cases the HLA genotype indicated an affected fetus, examination of the aborted fetuses was in agreement with the diagnosis. In one case an affected male fetus was diagnosed, the pregnancy is in progress. In seven cases an unaffected infant was predicted (four carriers and three homozygous normal infants).

Adrenal Gland Diseases↗
PubMed1978

Cytomegalic adrenal hypoplasia with pituitary cytomegaly.

A case of cytomegalic congenital adrenal hypoplasia (C.C.A.H.) is reported with similar cytomegalic changes in the hypophysis. In this case the pituitary gland was small as were the thyroid and testes. The various groups of patients with C.A.H. are presented. Associated pituitary cytomegaly does not appear to have been previously described, and this association between the adrenal and pituitary changes is discussed. It is suggested that the adrenal cytomegaly is the result of overstimulation by the pituitary and the similar change in the latter may be the result of overstimulation of a hypoplastic organ.

Adrenal Gland Diseases↗
PubMed1993

Prenatal adrenal hemorrhage confirmed by postnatal surgery.

A case is reported of massive adrenal hemorrhage in a newborn with evidence of bleeding in utero. A large cystic mass noted on prenatal ultrasonography at 20 weeks of gestation was followed by serial examinations until birth at 38 weeks of gestation. Postnatal evaluation included abdominal sonography, diethylenetriaminepentaacetic acid renal scan, voiding cystourethrography and abdominal plain film. Surgical exploration confirmed massive adrenal hemorrhage.

Adrenal Gland Diseases↗