{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T07:23:08Z","timestamp":1790752988033,"version":"4.1.0"},"reference-count":98,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2024,6,18]],"date-time":"2024-06-18T00:00:00Z","timestamp":1718668800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,16]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Primary pigmented nodular adrenocortical disease (PPNAD) and bilateral macronodular adrenocortical disease (BMAD) are 2 forms of adrenocortical nodular diseases causing Cushing's syndrome but are 2 very distinct conditions. PPNAD, affecting mostly young patients with an almost constant severe Cushing's syndrome, is characterized by pigmented micronodules, usually less than 1 cm, not always visible on imaging. On the contrary, BMAD is predominantly diagnosed in the fifth and sixth decades, with highly variable degrees of cortisol excess, from mild autonomous cortisol secretion to overt Cushing's syndrome. BMAD presents as large bilateral adrenal macronodules, easily observed on imaging. Both diseases are often genetically determined: frequently PPNAD is observed in a multiple neoplasia syndrome, Carney complex, and a germline genetic defect is identified in around 80% of index cases, always affecting key actors of the cAMP\/protein kinase A (PKA) pathway: mostly PRKAR1A, encoding the PKA 1-alpha regulatory subunit. On the other hand, BMAD appears mostly isolated, and 2 predisposing genes are known at present: ARMC5, accounting for around 20% of index cases, and the recently identified KDM1A, causing the rare presentation with food-dependent Cushing's syndrome, mediated by the ectopic expression of the glucose-dependent insulinotropic polypeptide receptor (GIPR) in adrenal nodules. GIPR was the first demonstrated receptor to illegitimately regulate cortisol secretion in nodular adrenocortical diseases, and a myriad of other receptors and paracrine signals were discovered afterward. The last 30 years were pivotal in the understanding of the genetics and pathophysiology of bilateral adrenocortical nodular diseases, leading to a personalized approach of these fascinating conditions.<\/jats:p>","DOI":"10.1210\/clinem\/dgae419","type":"journal-article","created":{"date-parts":[[2024,6,18]],"date-time":"2024-06-18T11:13:01Z","timestamp":1718709181000},"page":"2422-2432","source":"Crossref","is-referenced-by-count":14,"title":["Bilateral Adrenocortical Nodular Disease and Cushing's Syndrome"],"prefix":"10.1210","volume":"109","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1743-1598","authenticated-orcid":false,"given":"Lucas","family":"Bouys","sequence":"first","affiliation":[{"name":"Department of Endocrinology and National Reference Center for Rare Adrenal Diseases, H\u00f4pital Cochin, Assistance Publique H\u00f4pitaux de Paris , F-75014 Paris , France"},{"name":"Genomics and Signaling of Endocrine Tumors, Institut Cochin , INSERM U1016, CNRS UMR 8104, Universit\u00e9 Paris-Cit\u00e9, F-75014 Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7990-3434","authenticated-orcid":false,"given":"Florian","family":"Violon","sequence":"additional","affiliation":[{"name":"Genomics and Signaling of Endocrine Tumors, Institut Cochin , INSERM U1016, CNRS UMR 8104, Universit\u00e9 Paris-Cit\u00e9, F-75014 Paris , France"},{"name":"Department of Pathology, H\u00f4pital Cochin, Assistance Publique H\u00f4pitaux de Paris , F-75014 Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4723-095X","authenticated-orcid":false,"given":"Estelle","family":"Louiset","sequence":"additional","affiliation":[{"name":"Adrenal and Gonadal Pathophysiology, Universit\u00e9 Rouen Normandie, INSERM, NorDiC UMR 1239 , F-76000 Rouen , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1282-8732","authenticated-orcid":false,"given":"Mathilde","family":"Sibony","sequence":"additional","affiliation":[{"name":"Genomics and Signaling of Endocrine Tumors, Institut Cochin , INSERM U1016, CNRS UMR 8104, Universit\u00e9 Paris-Cit\u00e9, F-75014 Paris , France"},{"name":"Department of Pathology, H\u00f4pital Cochin, Assistance Publique H\u00f4pitaux de Paris , F-75014 Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6181-9648","authenticated-orcid":false,"given":"Herv\u00e9","family":"Lefebvre","sequence":"additional","affiliation":[{"name":"Adrenal and Gonadal Pathophysiology, Universit\u00e9 Rouen Normandie, INSERM, NorDiC UMR 1239 , F-76000 Rouen , France"},{"name":"Department of Endocrinology, Diabetes and Metabolic Diseases, CHU Rouen , F-76000 Rouen , 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