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Adrenocortical Adenoma (adrenocortical + adenoma)
Selected AbstractsAnalysis of somatic APC mutations in rare extracolonic tumors of patients with familial adenomatous polyposis coliGENES, CHROMOSOMES AND CANCER, Issue 2 2004Hendrik Bläker Patients with familial adenomatous polyposis coli (FAP) carry heterozygous mutations of the APC gene. At a young age, these patients develop multiple colorectal adenomas that consistently display a second somatic mutation in the remaining APC wild-type allele. Inactivation of APC leads to impaired degradation of ,-catenin, thereby promoting continuous cell-cycle progression. The role of APC inactivation in rare extracolonic tumors of FAP patients has not been characterized sufficiently. Among tissue specimen from 174 patients with known APC germ-line mutations, we identified 8 tumors infrequently seen in FAP. To investigate the pathogenic role of APC pathway deregulation in these lesions, they were analyzed for second-hit somatic mutations in the mutational cluster region of the APC gene. Immunohistochemistry was performed to compare the expression pattern of ,-catenin to the mutational status of the APC gene. Exon 3 of the ,-catenin gene (CTNNB1) was analyzed for activating mutations to investigate alternative mechanisms of elevated ,-catenin concentration. Although CTNNB1 mutations were not observed, second somatic APC mutations were found in 4 of the 8 tumors: a uterine adenocarcinoma, a hepatocellular adenoma, an adrenocortical adenoma, and an epidermal cyst. These tumors showed an elevated concentration of ,-catenin. No APC mutations were seen in focal nodular hyperplasia of the liver, angiofibrolipoma, and seborrheic wart. This is the first study reporting second somatic APC mutations in FAP-associated uterine adenocarcinoma and epidermal cysts. Furthermore, our data strengthen a role for impaired APC function in the pathogenesis of adrenal and hepatic neoplasms in FAP patients. © 2004 Wiley-Liss, Inc. [source] Primitive neuroectodermal tumor of the adrenal glandINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2006SHUICHI KOMATSU Abstract, We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery. [source] Combined adrenal adenoma and myelolipoma in a patient with Cushing's syndrome: Case report and review of the literatureINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2004HIROSHI HISAMATSU Abstract Myelolipoma is an uncommon benign tumor of unknown etiology and adrenal myelolipoma is rarely associated with endocrine disorders. We report a 67-year-old woman with Cushing's syndrome due to left adrenal adenoma associated with myelolipoma. The patient underwent laparoscopic left adrenalectomy and pathological examination revealed an adrenocortical adenoma associated with myelolipoma. To the best of our knowledge, 25 cases of endocrine dysfunction associated with myelolipoma have been reported in the English and Japanese literature. We review and discuss the pathogenesis of adrenal myelolipoma. [source] Adrenal myelolipoma associated with adenomaINTERNATIONAL JOURNAL OF UROLOGY, Issue 5 2004FRANCESCA MANASSERO Abstract The association of an adrenal myelolipoma with a non-functioning adenoma is very rare. Herein, we report on such a case in an asymptomatic 64-year-old woman. To the best of our knowledge, there is only one other case of non-functioning adrenocortical adenoma associated with myelolipoma in the same gland. Furthermore, only two other adenomas (mineral corticoids and corticosteroid producing tumors) associated with myelolipomas have been reported, and both myelolipomas were less than 1 cm in size. In conclusion, the 8.5 cm myelolipoma in our case is the largest compared with the three previously reported ones. [source] Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literatureACTA PAEDIATRICA, Issue 6 2007Xiumin 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] ES07 MICROARRAY GENE EXPRESSION ANALYSIS OF HUMAN ADRENOCORTICAL TUMOURSANZ JOURNAL OF SURGERY, Issue 2007P. 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] Intraoperative haemodynamic stability in patients with phaeochromocytoma , minimally invasive vs conventional open surgeryCLINICAL ENDOCRINOLOGY, Issue 3 2006Dirk Weismann Summary Objective, There is conflicting evidence, whether or not minimally invasive adrenalectomy (MA) is associated with an increased perioperative cardiovascular instability in phaeochromocytomas compared to conventional open adrenalectomy (CA). Design and patients, In a retrospective analysis of 49 patients with phaeochromocytoma we compared 27 cases of MA to 22 cases of CA by assessing intraoperative haemodynamic parameters and perioperative complications. Patients undergoing MA for adrenocortical adenomas (aldosteronomas n = 15, inactive adenomas n = 13) served as controls. Additionally, we investigated the effect of phenoxybenzamine (POB) pretreatment on intraoperative cardiovascular stability in 42 patients (ranked by maximum daily POB-dose) by comparing the highest (n = 10) with the lowest (n = 10) POB dose quartile (0·32 ± 0·2 and 2·17 ± 0·6 mg/kg/day, P < 0·001). Results, In phaeochromocytomas we found no significant difference in intraoperative haemodynamic parameters or complications when comparing MA with CA. In comparison to adrenocortical adenomas, MA in phaeochromocytomas was associated with a significantly higher maximum systolic BP (188 ± 29 vs 154 ± 22 mmHg, P < 0·001), more frequent hypertensive episodes (1[0,4]vs 0[0,1], P < 0·001), more episodes of systolic BP > 200 mmHg (0[0,4]vs 0[0,1], P = 0·03) and a higher demand for intraoperative fluids (3194 ml vs 1750 ml, P < 0·001). Most haemodynamic parameters did not differ significantly between high-dose POB pretreatment and low-dose POB pretreatment, but high-dose POB pretreatment was associated with a significantly higher intraoperative heart rate (120 ± 19·5 vs 94 ± 15·2 min,1, P < 0·01). Conclusion, There is no significant difference in haemodynamic stability between MA and CA in phaeochromocytomas, but it is significantly inferior when compared to MA for cortical adenomas. We could not detect a beneficial effect of high-dose compared to low-dose POB pretreatment on intraoperative cardiovascular stability. [source] | ||||||||||