 |
 |
|
Home |
Current Issue |
Past Issues |
In the Clinic |
ACP Journal Club |
CME |
Collections |
Audio/Video |
Mobile |
Subscribe |
Tools |
Help |
ACP Online
|
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
4 May 1999 | Volume 130 Issue 9 | Pages 759-771
Adrenocortical masses are among the most common tumors in humans. However, only a small proportion of these tumors cause endocrine diseases (such as primary hyperaldosteronism, hypercortisolism, hyperandrogenism, or hyperestrogenism), and less than 1% are malignant. In recent years, several of the molecular and cellular mechanisms involved in adrenal tumorigenesis have been unraveled. As a result, alterations in intercellular communication, local production of growth factors and cytokines, and aberrant expression of ectopic receptors on adrenal tumor cells have been implicated in adrenal cell growth, hyperplasia, tumor formation, and autonomous hormone production. Genetic and chromosomal abnormalities, including several chromosomal loci and the genes coding for p53, p57, and insulin-like growth factor II, have been reported in adrenal tumors. In addition, chromosomal markers have been identified in several familial syndromes associated with adrenal tumors; these include menin, which is responsible for multiple endocrine neoplasia type I, and the hybrid gene that causes glucocorticoid-remediable hyperaldosteronism. Algorithms for endocrine testing and imaging procedures are now available to codify screening for, confirmation of, and differentiation of causes of primary hyperaldosteronism and the Cushing syndrome. Improved radiologic, computerized radiologic, and magnetic resonance imaging techniques, as well as selective catheterization studies, are useful in localizing adrenal tumors and in distinguishing between benign and malignant lesions and between functional and nonfunctional nodules. Finally, recent refinements in the field of minimally invasive general surgery have made laparoscopic adrenalectomy the method of choice for removing adrenal tumors; this type of surgery allows shorter hospital stays, lower morbidity rates, and faster recovery.
Author and Article Information
An edited summary of a Clinical Staff Conference held on 28 April 1998 at the National Institutes of Health, Bethesda, Maryland.
Authors who wish to cite a section of the conference and specifically indicate its author may use this example for the form of the reference: Stratakis CA. Molecular genetics of adrenocortical tumors, pp 761-764. In: Bornstein SR, moderator. Adrenocortical tumors: recent advances in basic concepts and clinical management. Ann Intern Med. 1999; 130:759-771.
Acknowledgments: The authors thank John Doppman, Monika Ehrhart-Bornstein, Holger Willenberg, and David Torpy for constructive advice and support.
Grant Support: By grants from the Mildred Scheel Stiftung (10-1070-Re I) and by a Heisenberg grant (DFG BO 1141/6-1) (Dr. Bornstein).
Requests for Reprints: Stefan R. Bornstein, MD, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N 262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862; e-mail bornstes{at}ccl.nichd.nih.gov.
Current Author Addresses: Dr. Bornstein: Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N 262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862.
Dr. Chrousos: Section on Pediatric Endocrinology, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N 262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862.
Dr. Stratakis: Unit on Genetics and Endocrinology, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N 262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862. NIH CONFERENCE
Adrenocortical Tumors: Recent Advances in Basic Concepts and Clinical Management
This article has been cited by other articles:
|
|
 |
|
  S. Vuorenoja, A. Rivero-Muller, A. J Ziecik, I. Huhtaniemi, J. Toppari, and N. A Rahman Targeted therapy for adrenocortical tumors in transgenic mice through their LH receptor by Hecate-human chorionic gonadotropin {beta} conjugate Endocr. Relat. Cancer, June 1, 2008; 15(2): 635 - 648. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. Kaji, J. A Carrasquillo, W M. Linehan, C. C Chen, G. Eisenhofer, P. A Pinto, E. W Lai, and K. Pacak The role of 6-[18F]fluorodopamine positron emission tomography in the localization of adrenal pheochromocytoma associated with von Hippel-Lindau syndrome Eur. J. Endocrinol., April 1, 2007; 156(4): 483 - 487. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Horvath, L. Mathyakina, Q. Vong, V. Baxendale, A. L. Y. Pang, W.-Y. Chan, and C. A. Stratakis Serial Analysis of Gene Expression in Adrenocortical Hyperplasia Caused by a Germline PRKAR1A Mutation J. Clin. Endocrinol. Metab., February 1, 2006; 91(2): 584 - 596. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D E Schteingart, G M Doherty, P G Gauger, T J Giordano, G D Hammer, M Korobkin, and F P Worden Management of patients with adrenal cancer: recommendations of an international consensus conference Endocr. Relat. Cancer, September 1, 2005; 12(3): 667 - 680. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. M Pinto, A. E. C. Billerbeck, M. C. B. V. Fragoso, B. B. Mendonca, and A. C. Latronico Deletion Mapping of Chromosome 17 in Benign and Malignant Adrenocortical Tumors Associated with the Arg337His Mutation of the p53 Tumor Suppressor Protein J. Clin. Endocrinol. Metab., May 1, 2005; 90(5): 2976 - 2981. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. R. Bornstein and P. J. Hornsby What Can We Learn from Gene Expression Profiling for Adrenal Tumor Management? J. Clin. Endocrinol. Metab., March 1, 2005; 90(3): 1900 - 1902. [Full Text] [PDF]
|
|
|
|
|
|
G. W. Wolkersdorfer, C. Marx, J. Brown, S. Schroder, M. Fussel, E. P. Rieber, E. Kuhlisch, G. Ehninger, and S. R. Bornstein Prevalence of HLA-DRB1 Genotype and Altered Fas/Fas Ligand Expression in Adrenocortical Carcinoma J. Clin. Endocrinol. Metab., March 1, 2005; 90(3): 1768 - 1774. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. de Fraipont, M. El Atifi, N. Cherradi, G. Le Moigne, G. Defaye, R. Houlgatte, J. Bertherat, X. Bertagna, P.-F. Plouin, E. Baudin, et al. Gene Expression Profiling of Human Adrenocortical Tumors Using Complementary Deoxyribonucleic Acid Microarrays Identifies Several Candidate Genes as Markers of Malignancy J. Clin. Endocrinol. Metab., March 1, 2005; 90(3): 1819 - 1829. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. J. Hornsby Aging of the Human Adrenal Cortex Sci. Aging Knowl. Environ., September 1, 2004; 2004(35): re6 - re6. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A.-M. Lefrancois-Martinez, J. Bertherat, P. Val, C. Tournaire, N. Gallo-Payet, D. Hyndman, G. Veyssiere, X. Bertagna, C. Jean, and A. Martinez Decreased Expression of Cyclic Adenosine Monophosphate-Regulated Aldose Reductase (AKR1B1) Is Associated with Malignancy in Human Sporadic Adrenocortical Tumors J. Clin. Endocrinol. Metab., June 1, 2004; 89(6): 3010 - 3019. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Mansmann, J. Lau, E. Balk, M. Rothberg, Y. Miyachi, and S. R. Bornstein The Clinically Inapparent Adrenal Mass: Update in Diagnosis and Management Endocr. Rev., April 1, 2004; 25(2): 309 - 340. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Mannelli, P. Ferruzzi, P. Luciani, C. Crescioli, L. Buci, G. Corona, M. Serio, and A. Peri Cushing's Syndrome in a Patient with Bilateral Macronodular Adrenal Hyperplasia Responding to Cisapride: An in Vivo and in Vitro Study J. Clin. Endocrinol. Metab., October 1, 2003; 88(10): 4616 - 4622. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Bertherat, L. Groussin, F. Sandrini, L. Matyakhina, T. Bei, S. Stergiopoulos, T. Papageorgiou, I. Bourdeau, L. S. Kirschner, C. Vincent-Dejean, et al. Molecular and Functional Analysis of PRKAR1A and its Locus (17q22-24) in Sporadic Adrenocortical Tumors: 17q Losses, Somatic Mutations, and Protein Kinase A Expression and Activity Cancer Res., September 1, 2003; 63(17): 5308 - 5319. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Charmandari, G. Eisenhofer, S. L. Mehlinger, A. Carlson, R. Wesley, M. F. Keil, G. P. Chrousos, M. I. New, and D. P. Merke Adrenomedullary Function May Predict Phenotype and Genotype in Classic 21-Hydroxylase Deficiency J. Clin. Endocrinol. Metab., July 1, 2002; 87(7): 3031 - 3037. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. S. KIRSCHNER Signaling Pathways in Adrenocortical Cancer Ann. N.Y. Acad. Sci., June 1, 2002; 968(1): 222 - 239. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. P. Merke, S. R. Bornstein, N. A. Avila, and G. P. Chrousos Future Directions in the Study and Management of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency Ann Intern Med, February 19, 2002; 136(4): 320 - 334. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. A. Koch and G. P. Chrousos Is the Diminuto/Dwarf1 Gene Involved in Physiologic Adrenocortical Size Regulation and Tumor Formation? J. Clin. Endocrinol. Metab., November 1, 2001; 86(11): 5127 - 5129. [Full Text] [PDF]
|
|
|
|
|
|
N. Hiroi, G. P. Chrousos, B. Kohn, A. Lafferty, M. Abu-Asab, S. Bonat, A. White, and S. R. Bornstein Adrenocortical-Pituitary Hybrid Tumor Causing Cushing's Syndrome J. Clin. Endocrinol. Metab., June 1, 2001; 86(6): 2631 - 2637. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Lacroix, N. N'Diaye, J. Tremblay, and P. Hamet Ectopic and Abnormal Hormone Receptors in Adrenal Cushing's Syndrome Endocr. Rev., February 1, 2001; 22(1): 75 - 110. [Abstract] [Full Text]
|
|
|
|
 |
|
 S. R. Bornstein, H. Tian, A. Haidan, A. Bottner, N. Hiroi, G. Eisenhofer, S. M. McCann, G. P. Chrousos, and S. Roffler-Tarlov Deletion of tyrosine hydroxylase gene reveals functional interdependence of adrenocortical and chromaffin cell system in vivo PNAS, December 19, 2000; 97(26): 14742 - 14747. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. L. Phillips, M. M. Walther, J. C. Pezzullo, W. Rayford, P. L. Choyke, A. A. Berman, W. M. Linehan, J. L. Doppman, and J. R. Gill Jr. Predictive Value of Preoperative Tests in Discriminating Bilateral Adrenal Hyperplasia from an Aldosterone-Producing Adrenal Adenoma J. Clin. Endocrinol. Metab., December 1, 2000; 85(12): 4526 - 4533. [Abstract] [Full Text]
|
|
|
|
|
|
F. de Fraipont, M. El Atifi, C. Gicquel, X. Bertagna, E. M. Chambaz, and J. J. Feige Expression of the Angiogenesis Markers Vascular Endothelial Growth Factor-A, Thrombospondin-1, and Platelet-Derived Endothelial Cell Growth Factor in Human Sporadic Adrenocortical Tumors: Correlation with Genotypic Alterations J. Clin. Endocrinol. Metab., December 1, 2000; 85(12): 4734 - 4741. [Abstract] [Full Text]
|
|
|
|
 |
|
 A. R Lafferty, D. J Torpy, M. Stowasser, S. E Taymans, J. P. Lin, P. Huggard, R. D Gordon, and C. A Stratakis A novel genetic locus for low renin hypertension: familial hyperaldosteronism type II maps to chromosome 7 (7p22) J. Med. Genet., November 1, 2000; 37(11): 831 - 835. [Abstract] [Full Text]
|
|
|
|
|
|
H. Mircescu, J. Jilwan, N. N'Diaye, I. Bourdeau, J. Tremblay, P. Hamet, and A. Lacroix Are Ectopic or Abnormal Membrane Hormone Receptors Frequently Present in Adrenal Cushing's Syndrome? J. Clin. Endocrinol. Metab., October 1, 2000; 85(10): 3531 - 3536. [Abstract] [Full Text]
|
|
|
|
|
|
A. R. Ayala, S. Basaria, R. Udelsman, W. H. Westra, and G. S. Wand Corticotropin-Independent Cushing's Syndrome Caused by an Ectopic Adrenal Adenoma J. Clin. Endocrinol. Metab., August 1, 2000; 85(8): 2903 - 2906. [Abstract] [Full Text]
|
|
|
|
|
|
S. A. Murray, K. Davis, L. M. Fishman, and S. R. Bornstein {alpha}1 Connexin 43 Gap Junctions Are Decreased in Human Adrenocortical Tumors J. Clin. Endocrinol. Metab., February 1, 2000; 85(2): 890 - 895. [Abstract] [Full Text]
|
|
|
|
|
|
K.-M. Schulte, M. Mengel, M. Heinze, D. Simon, S. Scheuring, K. Köhrer, and H.-D. Röher Complete Sequencing and Messenger Ribonucleic Acid Expression Analysis of the MEN I Gene in Adrenal Cancer J. Clin. Endocrinol. Metab., January 1, 2000; 85(1): 441 - 448. [Abstract] [Full Text]
|
|
|
|
|
|
C. A. Stratakis, N. Sarlis, L. S. Kirschner, J. A. Carney, J. L. Doppman, L. K. Nieman, G. P. Chrousos, and D. A. Papanicolaou Paradoxical Response to Dexamethasone in the Diagnosis of Primary Pigmented Nodular Adrenocortical Disease Ann Intern Med, October 19, 1999; 131(8): 585 - 591. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. R. BORNSTEIN, T. TAJIMA, G. EISENHOFER, A. HAIDAN, and G. AGUILERA Adrenomedullary function is severely impaired in 21-hydroxylase-deficient mice FASEB J, July 1, 1999; 13(10): 1185 - 1194. [Abstract] [Full Text]
|
|
Article
