L. Groussin, L. Kirschner, C. Vincent-dejean, K. Perlemoine, E. Jullian et al., Molecular Analysis of the Cyclic AMP-Dependent Protein Kinase A (PKA) Regulatory Subunit 1A (PRKAR1A) Gene in Patients with Carney Complex and Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Reveals Novel Mutations and Clues For Pathophysiology: Augmented PKA Signaling is Associated with Adrenal Tumorigenesis in PPNAD, The American Journal of Human Genetics, vol.71, issue.6, pp.1433-4210344579, 1086.
DOI : 10.1086/344579

L. Kirschner, J. Carney, S. Pack, S. Taymans, C. Giatzakis et al., Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex, Nat Genet, vol.26, pp.89-921079238, 1038.

M. Fragoso, S. Domenice, A. Latronico, R. Martin, M. Pereira et al., Gene, The Journal of Clinical Endocrinology & Metabolism, vol.88, issue.5, pp.2147-512002, 2003.
DOI : 10.1210/jc.2002-021362

A. Horvath, S. Boikos, C. Giatzakis, A. Robinson-white, L. Groussin et al., A genome-wide scan identifies mutations in the gene encoding phosphodiesterase 11A4 (PDE11A) in individuals with adrenocortical hyperplasia, Nature Genetics, vol.85, issue.7, pp.794-80010, 1038.
DOI : 10.1038/ng1809

A. Horvath, C. Giatzakis, K. Tsang, E. Greene, P. Osorio et al., A cAMP-specific phosphodiesterase (PDE8B) that is mutated in adrenal hyperplasia is expressed widely in human and mouse tissues: a novel PDE8B isoform in human adrenal cortex, European Journal of Human Genetics, vol.60, issue.10, pp.1245-5385, 2008.
DOI : 10.1210/jc.2005-2383

A. Lacroix, E. Bolté, J. Tremblay, J. Dupré, P. Poitras et al., Gastric Inhibitory Polypeptide???Dependent Cortisol Hypersecretion ??? A New Cause of Cushing's Syndrome, New England Journal of Medicine, vol.327, issue.14, pp.974-80, 1992.
DOI : 10.1056/NEJM199210013271402

Y. Reznik, V. Allali-zerah, J. Chayvialle, R. Leroyer, P. Leymarie et al., Food-Dependent Cushing's Syndrome Mediated by Aberrant Adrenal Sensitivity to Gastric Inhibitory Polypeptide, New England Journal of Medicine, vol.327, issue.14, pp.981-987, 1992.
DOI : 10.1056/NEJM199210013271403

A. Lacroix, I. Bourdeau, A. Lampron, T. Mazzuco, J. Tremblay et al., Aberrant G-protein coupled receptor expression in relation to adrenocortical overfunction, Clinical Endocrinology, vol.73, 2010.
DOI : 10.1111/j.1365-2265.2009.03689.x

N. Miyamura, T. Taguchi, Y. Murata, K. Taketa, S. Iwashita et al., Inherited Adrenocorticotropin-Independent Macronodular Adrenal Hyperplasia with Abnormal Cortisol Secretion by Vasopressin and Catecholamines: Detection of the Aberrant Hormone Receptors on Adrenal Gland, Endocrine, vol.19, issue.3, pp.319-345, 2002.
DOI : 10.1385/ENDO:19:3:319

L. Lefèvre, J. Bertherat, and B. Ragazzon, Adrenocortical Growth and Cancer, Compr Physiol, vol.142, issue.Suppl 2, pp.293-326, 2015.
DOI : 10.1002/cphy.c140010

A. Lerario, A. Moraitis, and G. Hammer, Genetics and epigenetics of adrenocortical tumors, Molecular and Cellular Endocrinology, vol.386, issue.1-2, pp.67-84, 2014.
DOI : 10.1016/j.mce.2013.10.028

R. Libé and J. Bertherat, Molecular genetics of adrenocortical tumours, from familial to sporadic diseases, European Journal of Endocrinology, vol.153, issue.4, pp.477-87, 2005.
DOI : 10.1530/eje.1.02004

F. Tissier, C. Cavard, L. Groussin, K. Perlemoine, G. Fumey et al., Mutations of beta-catenin in adrenocortical tumors: activation of the Wnt signaling pathway is a frequent event in both benign and malignant adrenocortical tumors, Cancer Res, vol.65, pp.7622-7629, 2005.

F. Beuschlein, M. Fassnacht, G. Assié, D. Calebiro, C. Stratakis et al., Constitutive Activation of PKA Catalytic Subunit in Adrenal Cushing's Syndrome, New England Journal of Medicine, vol.370, issue.11, pp.1019-1047, 2014.
DOI : 10.1056/NEJMoa1310359

G. Assié, R. Libé, S. Espiard, M. Rizk-rabin, A. Guimier et al., Mutations in Macronodular Adrenal Hyperplasia with Cushing's Syndrome, New England Journal of Medicine, vol.369, issue.22, pp.2105-2119, 2013.
DOI : 10.1056/NEJMoa1304603

G. Assié, E. Letouzé, M. Fassnacht, A. Jouinot, W. Luscap et al., Integrated genomic characterization of adrenocortical carcinoma, Nature Genetics, vol.11, issue.6, pp.607-619, 2014.
DOI : 10.1038/ng.2953

R. Libé, G. Mantovani, S. Bondioni, A. Lania, C. Pedroni et al., and Gs?? in Sporadic Adrenocortical Tumors, Experimental and Clinical Endocrinology & Diabetes, vol.113, issue.5, pp.248-51, 2005.
DOI : 10.1055/s-2005-837651

J. Bertherat, L. Groussin, F. Sandrini, L. Matyakhina, T. Bei 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, vol.63, pp.5308-5327, 2003.

S. Bonnet, S. Gaujoux, P. Launay, C. Baudry, I. Chokri et al., Wnt/??-Catenin Pathway Activation in Adrenocortical Adenomas Is Frequently due to Somatic CTNNB1-Activating Mutations, Which Are Associated with Larger and Nonsecreting Tumors: A Study in Cortisol-Secreting and -Nonsecreting Tumors, The Journal of Clinical Endocrinology & Metabolism, vol.96, issue.2, pp.419-262010, 2011.
DOI : 10.1210/jc.2010-1885

M. Tadjine, A. Lampron, L. Ouadi, and I. Bourdeau, Frequent mutations of beta-catenin gene in sporadic secreting adrenocortical adenomas, Clin Endocrinol (Oxf), vol.68, pp.264-70, 2008.

Y. Cao, M. He, Z. Gao, Y. Peng, Y. Li et al., Activating hotspot L205R mutation in PRKACA and adrenal Cushing's syndrome, Science, vol.344, issue.6186, pp.913-920, 2014.
DOI : 10.1126/science.1249480

D. Dalmazi, G. Kisker, C. Calebiro, D. Mannelli, M. Canu et al., Novel Somatic Mutations in the Catalytic Subunit of the Protein Kinase A as a Cause of Adrenal Cushing's Syndrome: A European Multicentric Study, The Journal of Clinical Endocrinology & Metabolism, vol.99, issue.10, pp.2093-1002014, 2014.
DOI : 10.1210/jc.2014-2152

G. Goh, U. Scholl, J. Healy, M. Choi, M. Prasad et al., Recurrent activating mutation in PRKACA in cortisol-producing adrenal tumors, Nature Genetics, vol.110, issue.6, pp.613-620, 2014.
DOI : 10.1038/ng.2956

Y. Sato, S. Maekawa, R. Ishii, M. Sanada, T. Morikawa et al., Recurrent somatic mutations underlie corticotropin-independent Cushing's syndrome, Science, vol.344, issue.6186, pp.917-937, 2014.
DOI : 10.1126/science.1252328

J. Yang, S. Garrod, M. Deal, G. Anand, V. Woods et al., Allosteric Network of cAMP-dependent Protein Kinase Revealed by Mutation of Tyr204 in the P+1 Loop, Journal of Molecular Biology, vol.346, issue.1, pp.191-201, 2005.
DOI : 10.1016/j.jmb.2004.11.030

D. Calebiro, A. Hannawacker, S. Lyga, K. Bathon, U. Zabel et al., PKA catalytic subunit mutations in adrenocortical Cushing???s adenoma impair association with the regulatory subunit, Nature Communications, vol.211, 2014.
DOI : 10.1038/ncomms6680

F. Faucz, M. Zilbermint, M. Lodish, E. Szarek, G. Trivellin et al., ) Gene: A Clinical and Genetic Investigation, The Journal of Clinical Endocrinology & Metabolism, vol.99, issue.6, pp.1113-1122, 2014.
DOI : 10.1210/jc.2013-4280

G. Alencar, A. Lerario, M. Nishi, B. Mariani, M. Almeida et al., Mutations Are a Frequent Cause of Primary Macronodular Adrenal Hyperplasia, The Journal of Clinical Endocrinology & Metabolism, vol.99, issue.8, pp.1501-1510, 2014.
DOI : 10.1210/jc.2013-4237

L. Gagliardi, A. Schreiber, C. Hahn, J. Feng, T. Cranston et al., Mutations Are Common in Familial Bilateral Macronodular Adrenal Hyperplasia, The Journal of Clinical Endocrinology & Metabolism, vol.99, issue.9, pp.1784-92102014, 1210.
DOI : 10.1210/jc.2014-1265

U. Elbelt, A. Trovato, M. Kloth, E. Gentz, R. Finke et al., Tumor Syndrome: Concurrent Inactivating Germline and Somatic Mutations Are Associated With Both Primary Macronodular Adrenal Hyperplasia and Meningioma, The Journal of Clinical Endocrinology & Metabolism, vol.100, issue.1, pp.119-282014, 2015.
DOI : 10.1210/jc.2014-2648

S. Espiard, L. Drougat, R. Libé, G. Assié, K. Perlemoine et al., ARMC5 mutations in a large cohort of primary macronodular adrenal hyperplasia: clinical and functional consequences, J Clin Endocrinol Metab, pp.102014-4204, 1210.

M. Zilbermint, P. Xekouki, F. Faucz, A. Berthon, A. Gkourogianni et al., Primary aldosteronism and ARMC5 variants, J Clin Endocrinol Metab, pp.102014-4167, 1210.

M. Kirschner, R. Powell, and M. Lipsett, Cushing's Syndrome: Nodular Cortical Hyperplasia of Adrenal Glands with Clinical and Pathological Features Suggesting Adrenocortical Tumor, The Journal of Clinical Endocrinology & Metabolism, vol.24, issue.10, pp.947-55, 1964.
DOI : 10.1210/jcem-24-10-947

J. Bertherat, V. Contesse, E. Louiset, G. Barrande, C. Duparc et al., Screening for Illegitimate Receptors in Adrenocorticotropin-Independent Macronodular Adrenal Hyperplasia Causing Cushing???s Syndrome: Identification of Two Cases of Gonadotropin/Gastric Inhibitory Polypeptide-Dependent Hypercortisolism, The Journal of Clinical Endocrinology & Metabolism, vol.90, issue.3, pp.1302-102004, 2005.
DOI : 10.1210/jc.2004-1256

E. Louiset, V. Contesse, L. Groussin, D. Cartier, C. Duparc et al., Expression of vasopressin receptors in ACTH-independent macronodular bilateral adrenal hyperplasia causing Cushing's syndrome: molecular, immunohistochemical and pharmacological correlates, Journal of Endocrinology, vol.196, issue.1, pp.1-9, 2008.
DOI : 10.1677/JOE-07-0413

E. Louiset, C. Duparc, J. Young, S. Renouf, T. Nomigni et al., Intraadrenal corticotropin in bilateral macronodular adrenal hyperplasia Expression of ACTH receptor pathway genes in glucose-dependent insulinotrophic peptide (GIP)-dependent Cushing's syndrome, N Engl J Med Clin Endocrinol (Oxf), vol.369, issue.64, pp.2115-2529, 2006.

G. Assie, E. Louiset, N. Sturm, F. René-corail, L. Groussin et al., Systematic Analysis of G Protein-Coupled Receptor Gene Expression in Adrenocorticotropin-Independent Macronodular Adrenocortical Hyperplasia Identifies Novel Targets for Pharmacological Control of Adrenal Cushing???s Syndrome, The Journal of Clinical Endocrinology & Metabolism, vol.95, issue.10, pp.253-6210, 1210.
DOI : 10.1210/jc.2009-2281

T. Else, A. Kim, A. Sabolch, V. Raymond, A. Kandathil et al., Adrenocortical Carcinoma, Endocrine Reviews, vol.35, issue.2, pp.282-326, 2014.
DOI : 10.1210/er.2013-1029

G. Abiven, J. Coste, L. Groussin, P. Anract, F. Tissier et al., Clinical and Biological Features in the Prognosis of Adrenocortical Cancer: Poor Outcome of Cortisol-Secreting Tumors in a Series of 202 Consecutive Patients, The Journal of Clinical Endocrinology & Metabolism, vol.91, issue.7, pp.2650-2655, 2005.
DOI : 10.1210/jc.2005-2730

B. Allolio and M. Fassnacht, Adrenocortical Carcinoma: Clinical Update, The Journal of Clinical Endocrinology & Metabolism, vol.91, issue.6, pp.2027-2064, 2005.
DOI : 10.1210/jc.2005-2639

R. Libè, A. Fratticci, and J. Bertherat, Adrenocortical cancer: pathophysiology and clinical management, Endocrine Related Cancer, vol.14, issue.1, 2007.
DOI : 10.1677/erc.1.01130

J. Luton, S. Cerdas, L. Billaud, G. Thomas, B. Guilhaume et al., Clinical Features of Adrenocortical Carcinoma, Prognostic Factors, and the Effect of Mitotane Therapy, New England Journal of Medicine, vol.322, issue.17, pp.1195-201, 1990.
DOI : 10.1056/NEJM199004263221705

M. Fassnacht, M. Terzolo, B. Allolio, E. Baudin, H. Haak et al., Combination Chemotherapy in Advanced Adrenocortical Carcinoma, New England Journal of Medicine, vol.366, issue.23, pp.2189-97, 2012.
DOI : 10.1056/NEJMoa1200966

S. Gaujoux, S. Grabar, M. Fassnacht, B. Ragazzon, P. Launay et al., ??-Catenin Activation Is Associated with Specific Clinical and Pathologic Characteristics and a Poor Outcome in Adrenocortical Carcinoma, Clinical Cancer Research, vol.17, issue.2, pp.328-364, 2011.
DOI : 10.1158/1078-0432.CCR-10-2006

B. Ragazzon, R. Libé, S. Gaujoux, G. Assié, A. Fratticci et al., Transcriptome Analysis Reveals that p53 and ??-Catenin Alterations Occur in a Group of Aggressive Adrenocortical Cancers, Cancer Research, vol.70, issue.21, pp.8276-81, 2010.
DOI : 10.1158/0008-5472.CAN-10-2014

H. Hao, Y. Xie, Y. Zhang, O. Charlat, E. Oster et al., ZNRF3 promotes Wnt receptor turnover in an R-spondin-sensitive manner, Nature, vol.39, issue.7397, pp.195-20010, 1038.
DOI : 10.1038/nature11019

B. Koo, M. Spit, I. Jordens, T. Low, D. Stange et al., Tumour suppressor RNF43 is a stem-cell E3 ligase that induces endocytosis of Wnt receptors, Nature, vol.37, issue.7413, pp.665-674, 2012.
DOI : 10.1038/nature11308

Y. Xie, R. Zamponi, O. Charlat, M. Ramones, S. Swalley et al., Interaction with both ZNRF3 and LGR4 is required for the signalling activity of R-spondin, EMBO reports, vol.3, issue.12, pp.1120-1126, 2013.
DOI : 10.1038/embor.2013.167

D. Lau, W. Peng, W. Gros, P. Clevers, and H. , The R-spondin/Lgr5/Rnf43 module: regulator of Wnt signal strength, Genes & Development, vol.28, issue.4, pp.305-321, 2014.
DOI : 10.1101/gad.235473.113

K. Nord, J. Nilsson, E. Arbajian, F. Vult-von-steyern, O. Brosjö et al., Recurrent Chromosome 22 Deletions in Osteoblastoma Affect Inhibitors of the Wnt/Beta-Catenin Signaling Pathway, PLoS ONE, vol.13, issue.11, 2013.
DOI : 10.1371/journal.pone.0080725.s003