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Adrenal Tumours (adrenal + tumour)

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Medical Sciences	100%

Selected Abstracts

Phaeochromocytoma, new genes and screening strategies

CLINICAL ENDOCRINOLOGY, Issue 6 2006
Anne-Paule Gimenez-Roqueplo
Summary Following recent advances in the genetics of phaeochromocytomas and paragangliomas, the members of the European Network for the Study of Adrenal Tumours (ENS@T) Phaeochromocytoma Working Group have decided to share their genotyping data and to propose European recommendations for phaeochromocytoma/functional paraganglioma (PH/FPGL) genetic testing. Germline DNA from 642 patients was analysed by ENS@T teams. In 166 patients (25·9%) the disease was familial and caused by germline mutations in VHL (56), SDHB (34), SDHD (31), RET (31) or NF1 (14), causing von Hippel-Lindau disease, SDHB- or SDHD-PH/FPGL syndromes, multiple endocrine neoplasia type 2 (MEN 2) and type 1 neurofibromatosis (NF1), respectively. In almost 60% of inherited cases it was possible to formulate a probable genetic diagnosis based on family history and/or typical syndromic presentation. Genetic testing revealed mutations in 12·7% of cases with an apparently sporadic presentation. Several clinical characteristics, such as young age at onset, the presence of bilateral, extra-adrenal or multiple tumours or a malignant tumour, should be seen as indications for genetic testing. The ENS@T Phaeochromocytoma Working Group recommends the genetic testing of all patients with PH and FPGL and suggests a practice algorithm for the management of their exploration. [source]

ES07 MICROARRAY GENE EXPRESSION ANALYSIS OF HUMAN ADRENOCORTICAL TUMOURS

ANZ JOURNAL OF SURGERY, Issue 2007
P. S. H. Soon
Introduction Adrenal tumours are common, occurring in 7% of patients over the age of 50. Adrenocortical carcinomas, however, are rare, with an incidence of two per million population per year. The management of adrenocortical tumours is complex, compounded by the difficulty in discriminating benign from malignant tumours using conventional histology. A molecular marker which could reliably distinguish between the two groups would be valuable in patient management. Objectives The aim of this study was to identify molecular markers which will discriminate between adrenocortical carcinomas and adenomas using microarray gene expression analysis. Methods This study used RNA from 6 normal adrenal cortices, 16 adrenocortical adenomas and 12 carcinomas. Only samples with an RNA integrity number of 7.5 or greater were used. The samples were hybridised to Affymetrix HGU133plus2.0 genechips. Data analysis was performed with Partek and affylmgui softwares. Results Using a cutoff of B > 2 and M > 2 or <,2, 217 genes were found to be significantly differentially expressed between adrenocortical adenomas and carcinomas. Of these genes, 120 were unpregulated while 97 were downregulated. Seven of these genes have been selected for validation studies with real time reverse transcription polymerase chain reaction. Conclusion In this study, we found 217 genes which were significantly differentially expressed between adrenocortical adenomas and carcinomas. With validation and further studies, these genes will provide further insight into the pathogenesis of adrenocortical tumours as well as possibly proving to be reliable discriminators between adrenocortical adenomas and carcinomas. [source]

Retroperitoneoscopic partial adrenalectomy for small adrenal tumours (,1 cm): the Ruijin clinical experience in 88 patients

BJU INTERNATIONAL, Issue 6 2010
Xiao-jing Wang
Study Type , Therapy (case series) Level of Evidence 4 OBJECTIVE To present our experience of retroperitoneoscopic partial adrenalectomy (RPA) for small adrenal tumours, as with modern imaging methods small adrenal lesions are being diagnosed more commonly, and retroperitoneoscopic adrenal surgery for small adrenal tumours (,1 cm) can be challenging. PATIENTS AND METHODS We retrospectively reviewed the records of 389 consecutive retroperitoneoscopic adrenalectomies from September 2005 to December 2008, 88 of which were small adrenal tumours and treated by RPA. Ultrasonography and computed tomography (CT) were used in all patients before RPA, and magnetic resonance imaging or positron emission tomography/CT in some patients. We used RPA for adrenal tumours and total adrenalectomy for adrenal cancer. During the surgery, the internal part of the adrenal gland close to the retroperitoneum was freed first, and the whole adrenal tissue was dissected completely. The preoperative imaging was important in these procedures. RESULTS There were no deaths; conversions to open surgery were necessary in four patients (4.5%), the reasons being a missing target in two, massive haemorrhage caused by central adrenal vein injury in one, and severe adhesion in one. The mean (range) size of the adrenal tumours was 0.7 (0.5,1.0) cm, including 69 aldosterone-producing adenomas, 11 nonfunctional adrenal adenomas, three Cushing syndrome, two phaeochromocytomas, two myelolipomas and one melanoma. The operative duration in the initial 38 cases was significantly longer than that in the subsequent 50 (P < 0.01). However, there was no significant correlation between estimated blood loss and the number of procedures. Tumour size did not correlate with estimated blood loss and operative duration. There was no significant correlation between body mass index and operative duration. CONCLUSION RPA is a safe, effective and minimally invasive therapeutic option for patients with small adrenal tumours. With improved operative technique the RPA has been completed in more quickly. Freeing the internal part of the adrenal gland close to the retroperitoneum first, and exploring the whole adrenal tissue during surgery are the key points of RPA. The location of the small adrenal tumour can be different from that shown on imaging before surgery, and the abnormality of the adrenal gland should be considered. [source]

The use of three-dimensional computed tomography for assessing patients before laparoscopic adrenal-sparing surgery

BJU INTERNATIONAL, Issue 5 2006
Michael Mitterberger
OBJECTIVE To evaluate the efficacy of three-dimensional computed tomography (3D-CT) in delineating the relationship of the adrenal mass to adjacent normal structures in preparation for laparoscopic partial adrenalectomy. PATIENTS AND METHODS Multislice CT (1 mm slices, 0.5 s rotation time) was used to evaluate 12 patients before adrenal-sparing surgery for aldosterone-producing adenoma or phaeochromocytoma. The CT data were reconstructed using two rendering techniques; (i) volume rendering with the modified VOLREN software (Johns Hopkins Hospital, Baltimore, MD, USA) which allowed interactive 3D examination of the whole data volume within a few minutes; (ii) surface representations only of the interesting structures (kidney, adrenal tumour, vessels) represented in different colours and depicted together in a 3D scene using the software package 3DVIEWNIX. RESULTS In all, 14 adrenal masses in 12 patients were evaluated with 3D-CT; the number and location of lesions was accurate in all cases with both rendering techniques. The coloured surface-rendered images showed a consistently better delineation of the adrenal tumour from the normal tissue than did the volume-rendering technique. From this information all laparoscopic partial adrenalectomies could be completed as planned. CONCLUSIONS Interactive visualization of volume-rendered CT images was helpful for the planning and successful performance of the procedure, but coloured surface-rendered CT provided more convenient, immediate and accurate intraoperative information. [source]

Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature

ACTA PAEDIATRICA, Issue 6 2007
Xiumin Wang
Abstract Aim: To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension. Methods: The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome. Results: Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension-associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11,530 g) and the size ranged from 1.5 × 1.5 to 12 × 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non-functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow-up time of 3.5 years. Conclusions: Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non-functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours. [source]

Reoperative adrenal surgery: lessons learnt

ANZ JOURNAL OF SURGERY, Issue 5 2009
Charles T. Tan
With the widespread use of abdominal imaging, the detection and therefore incidence of adrenal tumours is increasing. The laparoscopic approach to primary surgical resection of adrenal tumours has now become the standard of care. There is scarce published literature regarding the management and outcomes of recurrent adrenal tumours. The aim of the present study was therefore to review the authors' experience with reoperative adrenal surgery. A retrospective review of reoperative adrenalectomy cases identified from the prospectively maintained University of Sydney Endocrine Surgical Unit Database from January 1988 to July 2007 was carried out. There were nine (3.5%) reoperative adrenalectomies in six patients. Two were cases of adrenocortical carcinoma, two involved cases of familial phaeochromocytomas and two cases were due to sporadic phaeochromocytomas. Reoperative adrenal surgery is an uncommon event. During the index surgery for adrenal tumours, all adrenal tissue should be removed and knowledge of the vagaries of adrenal anatomy is essential. Reoperative adrenal surgery is a safe procedure and may confer survival benefit or symptom relief. Lifelong follow up is essential for all patients who have had surgery for functional and malignant adrenal tumours. [source]

Retroperitoneoscopic partial adrenalectomy for small adrenal tumours (,1 cm): the Ruijin clinical experience in 88 patients

Retroperitoneal endoscopic adrenalectomy is safe and effective

BRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 11 2010
J. M. J. Schreinemakers
Background: The aim of this study was to review an experience with retroperitoneal endoscopic adrenalectomy (REA). This is the procedure of choice for adrenal tumours at this institution. Methods: Between 1997 and 2008, 112 REAs were performed in a single university centre. Data were retrieved retrospectively from a prospectively collected database, including information on patient demographics, surgical procedure, complications and hospital stay. Results: One hundred and twelve REAs were carried out successfully in 105 patients, including seven bilateral adrenalectomies. Thirty-nine patients with unilateral adrenal disease had a phaeochromocytoma, of whom 16 had multiple endocrine neoplasia syndrome type 2, 21 patients had Cushing's disease and 20 had Conn's disease. Median body mass index was 27 (interquartile range 23,29) kg/m2. The median duration of unilateral operations was 100 (90,130) min with a median blood loss of 5 ml. Median tumour size was 3·1 (2·0,4·4) cm. Conversion from REA to open surgery was needed in two patients. Seven patients experienced postoperative complications (2 major, 5 minor). One patient needed a reoperation. The median postoperative hospital stay was 3 days. A learning curve with a significant decrease in operating time was observed over the years. Conclusion: REA appears to be a safe and effective surgical technique for adrenal gland tumours up to 6 cm in diameter, with a minimal complication rate. Copyright © 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. [source]

Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature

Familial vasopressin-sensitive ACTH-independent macronodular adrenal hyperplasia (VPs-AIMAH): clinical studies of three kindreds

CLINICAL ENDOCRINOLOGY, Issue 6 2009
Lucia Gagliardi
Summary Objective, Cushing's syndrome due to familial ACTH-independent macronodular adrenal hyperplasia (AIMAH) has been reported in small kindreds. In vasopressin-sensitive AIMAH (VPs-AIMAH), VP stimulates an aberrant, ACTH-independent increase in cortisol. The aims of this study were to (i) delineate the preclinical phenotype of VPs-AIMAH in a three-generation kindred (AIMAH-01) and two smaller kindreds (AIMAH-02 and AIMAH-03) and (ii) investigate the aetiology of VP sensitivity in AIMAH-01. Design, Clinical studies of three kindreds for adrenal tumours or early Cushing's and molecular studies of adrenal tumours (AIMAH-01). Patients, Thirty-three individuals, from three kindreds, were screened for perturbations of the hypothalamic,pituitary,adrenal axis or adrenal tumours. Measurements, Patients underwent clinical, biochemical and adrenal imaging investigations. Evaluation included low-dose (1 IU/70 kg) VP stimulation. Adrenal VP receptor (AVPR1A, AVPR1B, AVPR2) expression (AIMAH-01) was assessed using RT-PCR and immunohistochemistry (IHC). IHC for VP was also performed. Results, AIMAH-01 had three siblings with Cushing's, and four individuals with suppressed ACTH/aberrant VP responses and/or adrenal nodules. In AIMAH-02, a father and son were affected. AIMAH-03 had three siblings with Cushing's. RT-PCR showed adrenal overexpression of AVPR1A and AVPR1B. IHC detected AVPR1A. The adrenal tumour from one patient also stained weakly for VP and AVPR2. Conclusion, Adrenal nodules, suppressed ACTH and increased VP sensitivity may represent preclinical disease, allowing early detection, and treatment, of affected individuals. In AIMAH-01, increased VP sensitivity may be due to adrenal VP receptor overexpression. In these kindreds, VPs-AIMAH is familial, and autosomal dominant inheritance is most likely. [source]

Clinical and biochemical characteristics of normotensive patients with primary aldosteronism: a comparison with hypertensive cases

CLINICAL ENDOCRINOLOGY, Issue 1 2008
Virginie Médeau
Summary Objective, It is unknown why some patients with biochemical evidence of primary aldosteronism (PA) do not develop hypertension. We aimed to compare clinical and biochemical characteristics of normotensive and hypertensive patients with PA. Design and patients, Retrospective comparison of 10 normotensive and 168 hypertensive patients with PA for office or ambulatory blood pressure, serum potassium, plasma aldosterone and renin concentrations; the aldosterone : renin ratio, and tumour size. Comparison of initial hormonal pattern and drop in blood pressure following adrenalectomy in five normotensive and nine hypertensive patients matched for age, sex and body mass index. Results, The 10 normotensive patients were women and presented with hypokalemia or an adrenal mass. Age, plasma aldosterone and renin concentrations were similar in normotensive and hypertensive cases, but kalemia and body mass index were significantly lower in the normotensive patients. Mean tumour diameter was larger in the normotensive patients than in the hypertensive matched patients with an adenoma (P < 0·01). In normotensive patients, diastolic blood pressure and upright aldosterone correlated negatively with kalemia. Blood pressure was lowered similarly after adrenalectomy in five normotensive PA patients and in their matched hypertensive counterparts. Aldosterone synthase expression was detected in four out of five adrenal tumours. Conclusions, Blood pressure may be normal in patients with well-documented PA. The occurrence of hypokalemia, despite a normal blood pressure profile, suggests that protective mechanisms against hypertension are present in normotensive patients. [source]

Immunohistochemical localization of somatostatin receptor subtypes in benign and malignant adrenal tumours

CLINICAL ENDOCRINOLOGY, Issue 6 2008
Nicole Unger
Summary Background, Somatostatin mediates its action through five receptor subtypes (sst1,5) that are widely distributed in various endocrine tissues and tumours. Because of the inhibitory effects of somatostatin, long-acting analogues have been synthesized. In contrast to their well-established use in neuroendocrine and pituitary tumours, little is known about their potential use in adrenal tumours. Objective, We examined somatostatin receptor protein expression in adrenal tumours of various aetiologies. Immunostaining was performed with specific polyclonal antibodies for sst1,5. Design, Seven benign and eight malignant pheochromocytomas (PHEOs), eight aldosterone-secreting adenomas (APAs), nine cortisol-secreting adenomas (CPAs), seven nonfunctioning adrenal tumours (NFAs) and 25 adrenal carcinomas (CAs) as well as eight normal adrenal glands were investigated. Measurements, Staining pattern, distribution and subcellular localization of the somatostatin receptor subtypes were evaluated. Results, In the majority of normal cortices the expression of sst1,5 was limited to the reticular zone. The medulla was predominantly positive for sst3. Most cortical adenomas were positive for all five subtypes. However, in the majority of these tumours, less than 30% of cells were positively stained. A high expression of sst4 was found in CPAs but only very few cortical carcinomas exhibited sst immunostaining. All benign PHEOs were positive for sst3. The majority presented with more than 60% of tumour cells stained. By contrast, only six out of eight malignant PHEOs were positive for sst3. Conclusions, Somatostatin receptor subtypes are expressed in PHEOs as well as in tumours of the adrenal cortex with tumour-specific distribution patterns. This may offer new diagnostic and therapeutic possibilities. [source]