Adrenocortical tumors

Figure 5. Y1 adrenocortical cells in culture. Y1 mouse adrenocortical tumor cells (a) in the absence and (b) in the presence of 1 mM 8-bromocyclic AMP overnight. (Photos courtesy of Margaret Wong and Dr. Bernard Schimmer.)...

Y1 Mouse adrenocortical tumor Adrenocortical cell Methyl sulfone metabolites of DDT and PCBs Inhibition of corticosterone synthesis by competitive inhibition of cytochrome P450... 

Both type I and type II cyclic AMP-dependent protein kinase forms are found in the adrenal cortex [8]. The predominant form in the Y1 adrenocortical tumor cell line is type I [2], Yl(Kin) mutants with RI subunits which have a much lower affinity for cyclic AMP lack ACTH-stimulated cyclic AMP production, thus demonstrating the involvement of the type I form of cyclic AMP-dependent protein kinase in the action of ACTH. 

Nonfunctioning Adrenocortical Tumors Approximately 2% of the population have an adrenal tumor most of these tumors are nonfunctioning and are sometimes called incidentalomas. They are found when CT scans of the abdomen are performed that can easily detect tumors 1 cm in diameter or 5 g in weight. No virilizing tumors smaller than 1 cm in diameter have been reported. Carcinomas are usually more than 30 g in weight. 

Adrenocortical tumors J Expression loss of heterozygosity in tumors [119, 120]... 

S. R. Bomstein, C. A. Stratakis, and G. P. Chrousos Adrenocortical tumors recent advances in basic concepts and clinical management. Annals of Internal Medicine 130,759 (1999). 

The distinction of benign and malignant adrenocortical tumors is often problematic by standard histopathologic... 

Over the past decade, there have been considerable advances in the understanding of both sporadic and heritable adrenocortical tumors at the molecular level. Genetic alterations present in familial tumors include mutations of p53 (Li-Eraumeni syndrome), menin (MENl) PRKARIA (Carney complex) and p57kip2 (CDNKIC), KCNQIOT, H19, and IGE-II overexpression in Beckwith-Weidemann. ... 

Adrenocortical tumors are positive for vimentin and are variably positive for cytokeratins. 

Melan A (A1 03), inhibin, and calretinin are useful for the distinction of adrenocortical tumors from other tumor types. 

Adrenocortical tumors are often positive for synaptophy-sin and neurofilaments. 

Adrenocortical tumors generally overexpress ICF-2 and cyclin-dependent kinase 4. 

Pheochromocytomas are positive for chromogranins and synaptophysin, while the expression of synaptophysin is characteristic of adrenocortical tumors. 

Schroder S, Padberg BC, Achilles E, et al. Immunocytochem-istry in adrenocortical tumors A clinicopathological study of 72 neoplasms. Virchows Arch A Pathol Anat Histopathol. 1992 420 65-70. 

Arola J, Liu J, Heikkila P, et al. Expression of inhibin alpha in adrenocortical tumors reflects the hormonal status of the neoplasm. / Endocrinol. 2000 165 223-229. 

Schmitt A, Saremaslani P, Schmid S, et al. IGF 11 and MlB-1 immunohistochemistry is helpful in the differentiation of benign from malignant adrenocortical tumors. Histopathology. 2006 49 298-307. 

Giordano TJ, Thomas DG, Kuiche R, et al. Distinct transcriptional profiles of adrenocortical tumors uncovered by microarray analysis. Am J Pathol. 2003 162 521-531. 

West AN, Neale GA, Pounds S, et al. Gene expression profiling of childhood adrenocortical tumors. Cancer Res. 2007 67 600-608. 

Chivite A, MatiasGuiu X, Pons G, et al. Inhibin A expression in adrenal neoplasms—A new immunohistochemical marker for adrenocortical tumors. Appl Immunohistochem. 1998 6 42-49. 

The oversecretion of hormone molecules is most often caused by a tumor. Several types of pituitary tumor cause endocrine diseases. For example, one of the most common causes of Cushing s disease is an abnormal proliferation of ACTH-producing cells. Cushing s disease is characterized by obesity, hypertension, and elevated blood glucose levels. Patients with Cushing s disease develop a characteristic appearance a puffy moon face and a buffalo hump caused by fat deposits between the shoulders. Occasionally, Cushing s disease is caused by adrenocortical tumors. 

Approximately half of the families with the Li-Fraumeni cancer syndrome carry one mutant p53 allele in somatic cells (Malkin et al., 1990 Srivastava et al., 1990 see review in Kleihues et al., 1997). This disease is characterized by familial clustering of various cancers, particularly early-onset breast cancer, sarcomas, leukemias, and brain and adrenocortical tumors. Mice with targeted nonfunctional p53 alleles provide an experimental model for this disorder. Early spontaneous tumors, primarily lymphomas and sarcomas, arise frequently in p53 / and p53+/ mice, reflecting the Li-Fraumeni syndrome in these respects. Curiously, mammary and brain tumors are uncommon in p53 knockout mice (Donehower, 1996b Eng et al., 1997). 

Squamous cell carcinoma, transitional cell carcinoma, granular cell tumor, adrenocortical tumors, ovarian sex cord stromal tumors... 

Synaptophysin Neuroendocrine tumors pituitary adenomas, medullary thyroid carcinoma, pheochromocytoma, islet cell tumors, small cell carcinoma, carcinoid and neuroendocrine Medulloblastoma, retinoblastoma, neurocytoma, ependymoma, neuroblastoma, adrenocortical tumors. Merkel cell tumors Neuronal and neuroendocrine cells, carotid body cells, adrenal cortex and medulla... 

Magnusson S, Gisselsson D, Wiebe T, Kristofferson U, Borg A, Olsson H. Prevalence of germline TP53 mutations and history of Li-Fraumeni syndrome in families with childhood adrenocortical tumors, choroid plexus tumors, and rhabdomyosarcoma a population-based survey. Pediatr Blood Cancer. 2012 50 846-53. 

Adrenocortical Tumors. Virilization, hypercortisolism, feminization, abdominal pain, palpable abdominal tumor, or combinations of these features are clinical symptoms of adrenocortical tumors. Both types of adrenocortical tumors (carcinoma and adenoma) can produce a wide variety of steroid hormones. This is a consequence of multiple enzyme deficiencies in tumor tissues. The tumor cells are capable of synthesizing large amounts of steroid hormone precursors independent of ACTFI stimulation. Excessively high amounts of DHEA and other 3p hydroxy-5-ene steroids characterize the urinary steroid profile in children with adrenocortical carcinoma, but similar profiles can also be produced by adrenal adenomas. Elevated lip hydroxy-androsterone excretion alone or combined with high excretion of cortisol metabolites or 3p hydroxy-5-ene steroids are characteristic of the urinary steroid profile for adrenocortical adenomas [36,37]. 

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