{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T16:00:17Z","timestamp":1789747217775,"version":"4.0.1"},"reference-count":99,"publisher":"Bioscientifica","issue":"6","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:p>Cancer of the adrenal cortex (ACC) is a rare endocrine malignancy with limited treatment options. Patients typically present with autonomous hormonal overproduction and\/or a large abdominal mass. Hormonal assays and medical imaging can be diagnostic, but urinary steroid profiling might be a more sensitive technique to assess malignancy in adrenal tumours. The stage of the disease at diagnosis is the most important prognostic factor. The current staging system needs refinement, especially to separate aggressive from indolent disease in stage IV patients and to select patients who need adjuvant treatment after complete surgical resection. Regarding the latter, assessing the proliferation index Ki-67 seems the best tool currently available. Genomic profiling is expected to become of clinical relevance in the future. Medical therapy is centred on the adrenolytic drug mitotane, which carries considerable toxicity and is not easy to manage. Its tolerability and long plasma level build-up phase may be improved by therapeutic drug monitoring based on pharmacokinetic modelling and intensive counselling of patients. Current chemotherapy regimens can offer disease stabilization in about 50% of patients, but an objective response should be expected in &lt;25%. Research on targeted therapy and immunotherapy is difficult in this rare disease with often heavily pre-treated patients and has not yet been successful. Quality of care should be ensured by treating patients in centres with established experience in multidisciplinary oncologic care, who adhere to prevailing guidelines and state-of-the-art in diagnostic and treatment concepts. International collaboration in fundamental research and clinical trials is the key to further elucidate the pathogenesis and to improve patient care.<\/jats:p>","DOI":"10.1530\/erc-15-0318","type":"journal-article","created":{"date-parts":[[2015,8,10]],"date-time":"2015-08-10T21:49:46Z","timestamp":1439243386000},"page":"R325-R338","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":35,"title":["Developing treatment for adrenocortical carcinoma"],"prefix":"10.1530","volume":"22","author":[{"given":"T M A","family":"Kerkhofs","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M H T","family":"Ettaieb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I G C","family":"Hermsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H R","family":"Haak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"2015110216263202000_22.6.R325.1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2005-2730"},{"key":"2015110216263202000_22.6.R325.2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2011-1565"},{"key":"2015110216263202000_22.6.R325.3","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2006-0706"},{"key":"2015110216263202000_22.6.R325.4","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2953"},{"key":"2015110216263202000_22.6.R325.5","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2012-2993"},{"key":"2015110216263202000_22.6.R325.6","doi-asserted-by":"publisher","DOI":"10.1210\/jcem-73-1-18"},{"key":"2015110216263202000_22.6.R325.7","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(20010915)92:6<1385::AID-CNCR1461>3.0.CO;2-2"},{"key":"2015110216263202000_22.6.R325.8","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdf291"},{"key":"2015110216263202000_22.6.R325.9","doi-asserted-by":"publisher","DOI":"10.1007\/s12672-011-0094-2"},{"key":"2015110216263202000_22.6.R325.10","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-53-4-672"},{"key":"2015110216263202000_22.6.R325.11","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19981115)83:10<2194::AID-CNCR19>3.0.CO;2-3"},{"key":"2015110216263202000_22.6.R325.12","doi-asserted-by":"publisher","DOI":"10.1677\/erc.1.01025"},{"key":"2015110216263202000_22.6.R325.13","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mds231"},{"key":"2015110216263202000_22.6.R325.14","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-11-0918"},{"key":"2015110216263202000_22.6.R325.15","doi-asserted-by":"publisher","DOI":"10.1016\/j.eururo.2013.11.006"},{"key":"2015110216263202000_22.6.R325.16","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2014-3182"},{"key":"2015110216263202000_22.6.R325.17","doi-asserted-by":"publisher","DOI":"10.1016\/j.eururo.2010.06.024"},{"key":"2015110216263202000_22.6.R325.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvir.2010.05.020"},{"key":"2015110216263202000_22.6.R325.19","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-13-0051"},{"key":"2015110216263202000_22.6.R325.20","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2012-2851"},{"key":"2015110216263202000_22.6.R325.21","doi-asserted-by":"publisher","DOI":"10.1007\/s00464-013-3034-0"},{"key":"2015110216263202000_22.6.R325.22","doi-asserted-by":"publisher","DOI":"10.1677\/ERC-08-0103"},{"key":"2015110216263202000_22.6.R325.23","doi-asserted-by":"publisher","DOI":"10.1097\/FPC.0b013e3283606cb2"},{"key":"2015110216263202000_22.6.R325.24","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2008.18.5678"},{"key":"2015110216263202000_22.6.R325.25","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-013-3164-6"},{"key":"2015110216263202000_22.6.R325.26","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-014-3944-7"},{"key":"2015110216263202000_22.6.R325.27","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2012-2559"},{"key":"2015110216263202000_22.6.R325.28","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-10-0956"},{"key":"2015110216263202000_22.6.R325.29","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2265.2005.02403.x"},{"key":"2015110216263202000_22.6.R325.30","doi-asserted-by":"publisher","DOI":"10.1016\/j.beem.2008.10.008"},{"key":"2015110216263202000_22.6.R325.31","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2006-1007"},{"key":"2015110216263202000_22.6.R325.32","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.24030"},{"key":"2015110216263202000_22.6.R325.33","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2010-0803"},{"key":"2015110216263202000_22.6.R325.34","doi-asserted-by":"publisher","DOI":"10.1038\/nrendo.2010.235"},{"key":"2015110216263202000_22.6.R325.35","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1200966"},{"key":"2015110216263202000_22.6.R325.36","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(15)70081-1"},{"key":"2015110216263202000_22.6.R325.37","doi-asserted-by":"publisher","DOI":"10.1186\/s40425-015-0047-3"},{"key":"2015110216263202000_22.6.R325.38","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMsa1010705"},{"key":"2015110216263202000_22.6.R325.39","doi-asserted-by":"publisher","DOI":"10.1007\/s12020-013-9878-1"},{"key":"2015110216263202000_22.6.R325.40","doi-asserted-by":"publisher","DOI":"10.1210\/jcem-32-2-192"},{"key":"2015110216263202000_22.6.R325.41","doi-asserted-by":"publisher","DOI":"10.1530\/EC-12-0028"},{"key":"2015110216263202000_22.6.R325.42","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-08-1067"},{"key":"2015110216263202000_22.6.R325.43","doi-asserted-by":"publisher","DOI":"10.1677\/erc.1.01124"},{"key":"2015110216263202000_22.6.R325.44","doi-asserted-by":"crossref","unstructured":"Grubbs EG Callender GG Xing Y Perrier ND Evans DB Phan AT Lee JE 2010 Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane. Annals of Surgical Oncology 17 263\u2013270. (doi:10.1245\/s10434-009-0716-x).","DOI":"10.1245\/s10434-009-0716-x"},{"key":"2015110216263202000_22.6.R325.45","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.1994.183"},{"key":"2015110216263202000_22.6.R325.46","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2012-2367"},{"key":"2015110216263202000_22.6.R325.47","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2011-2765"},{"key":"2015110216263202000_22.6.R325.48","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-07-0723"},{"key":"2015110216263202000_22.6.R325.49","doi-asserted-by":"publisher","DOI":"10.1007\/BF03346675"},{"key":"2015110216263202000_22.6.R325.50","doi-asserted-by":"publisher","DOI":"10.1016\/j.surg.2012.02.005"},{"key":"2015110216263202000_22.6.R325.51","unstructured":"Ho V de Raaf A van der Hoeven K Jansen-Landheer M 2013 IKNL berekent effect volumenormen op ziekenhuizen en pati\u00ebnten. Medisch Contact 1 34\u201337."},{"key":"2015110216263202000_22.6.R325.52","doi-asserted-by":"publisher","DOI":"10.1097\/PPO.0b013e31829e3221"},{"key":"2015110216263202000_22.6.R325.53","doi-asserted-by":"publisher","DOI":"10.1055\/s-0032-1331743"},{"key":"2015110216263202000_22.6.R325.54","doi-asserted-by":"publisher","DOI":"10.1007\/s00268-005-0329-x"},{"key":"2015110216263202000_22.6.R325.55","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2013.02.034"},{"key":"2015110216263202000_22.6.R325.56","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-13-0142"},{"key":"2015110216263202000_22.6.R325.57","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2013-2281"},{"key":"2015110216263202000_22.6.R325.58","doi-asserted-by":"publisher","DOI":"10.1007\/s12672-015-0224-3"},{"key":"2015110216263202000_22.6.R325.59","doi-asserted-by":"publisher","DOI":"10.1097\/FTD.0000000000000102"},{"key":"2015110216263202000_22.6.R325.60","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2265.2011.04214.x"},{"key":"2015110216263202000_22.6.R325.61","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2012-1419"},{"key":"2015110216263202000_22.6.R325.62","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdv329"},{"key":"2015110216263202000_22.6.R325.63","doi-asserted-by":"publisher","DOI":"10.1016\/j.surg.2012.08.014"},{"key":"2015110216263202000_22.6.R325.64","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1973.03220100011003"},{"key":"2015110216263202000_22.6.R325.65","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2009.12.007"},{"key":"2015110216263202000_22.6.R325.66","doi-asserted-by":"publisher","DOI":"10.1007\/BF02739705"},{"key":"2015110216263202000_22.6.R325.67","doi-asserted-by":"publisher","DOI":"10.1007\/s00268-010-0532-2"},{"key":"2015110216263202000_22.6.R325.68","doi-asserted-by":"publisher","DOI":"10.1016\/j.surg.2012.08.024"},{"key":"2015110216263202000_22.6.R325.69","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-012-2760-1"},{"key":"2015110216263202000_22.6.R325.70","doi-asserted-by":"publisher","DOI":"10.1007\/BF00258213"},{"key":"2015110216263202000_22.6.R325.71","doi-asserted-by":"publisher","DOI":"10.1507\/endocrj.K07-079"},{"key":"2015110216263202000_22.6.R325.72","doi-asserted-by":"publisher","DOI":"10.1111\/cen.12458"},{"key":"2015110216263202000_22.6.R325.73","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2013-1396"},{"key":"2015110216263202000_22.6.R325.74","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.24331"},{"key":"2015110216263202000_22.6.R325.75","doi-asserted-by":"publisher","DOI":"10.1016\/j.eururo.2010.01.036"},{"key":"2015110216263202000_22.6.R325.76","unstructured":"Pucci E, Papathomas TG, Giordano TJ, Lu H, Duregon E, Volante M, Papotti M, Lloyd R, Tischler A & Nose V 2014 An international Ki-67 reproducibility study in adrenocortical cancer: a multicenter interobserver variation analysis using virtual microscopy. Abstract on 13th scientific meeting of ENS@T, Nice, France"},{"key":"2015110216263202000_22.6.R325.77","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2007-2564"},{"key":"2015110216263202000_22.6.R325.78","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3182367ac3"},{"key":"2015110216263202000_22.6.R325.79","doi-asserted-by":"crossref","unstructured":"Saade N Sadler C Goldfarb M Impact of regional lymph node dissection on disease specific survival in adrenal cortical carcinoma Hormone and Metabolic Research 2015 [in press] doi:10.1055\/s-0035-1549877).","DOI":"10.1055\/s-0035-1549877"},{"key":"2015110216263202000_22.6.R325.80","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijrobp.2010.04.030"},{"key":"2015110216263202000_22.6.R325.81","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2014.55.9567"},{"key":"2015110216263202000_22.6.R325.82","doi-asserted-by":"publisher","DOI":"10.1007\/s12022-006-0006-0"},{"key":"2015110216263202000_22.6.R325.83","doi-asserted-by":"crossref","unstructured":"Sbiera S Kroiss M Thamm T Beyer M Majidi F Kuehner D Wobser M Becker JC Adam P Ronchi C 2013 Survivin in adrenocortical tumors \u2013 pathophysiological implications and therapeutic potential. Hormone and Metabolic Research 45 137\u2013146. (doi:10.1055\/s-0032-1327750).","DOI":"10.1055\/s-0032-1327750"},{"key":"2015110216263202000_22.6.R325.84","doi-asserted-by":"publisher","DOI":"10.1007\/s10434-999-0719-7"},{"key":"2015110216263202000_22.6.R325.85","doi-asserted-by":"publisher","DOI":"10.1007\/BF00265384"},{"key":"2015110216263202000_22.6.R325.86","doi-asserted-by":"publisher","DOI":"10.1016\/0039-128X(66)90132-2"},{"key":"2015110216263202000_22.6.R325.87","doi-asserted-by":"publisher","DOI":"10.1677\/ERC-09-0281"},{"key":"2015110216263202000_22.6.R325.88","doi-asserted-by":"publisher","DOI":"10.1007\/s00432-011-0988-y"},{"key":"2015110216263202000_22.6.R325.89","doi-asserted-by":"publisher","DOI":"10.1210\/jc.85.6.2234"},{"key":"2015110216263202000_22.6.R325.90","doi-asserted-by":"publisher","DOI":"10.1016\/S0090-4295(00)00852-9"},{"key":"2015110216263202000_22.6.R325.91","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa063360"},{"key":"2015110216263202000_22.6.R325.92","doi-asserted-by":"publisher","DOI":"10.1016\/j.surge.2013.01.004"},{"key":"2015110216263202000_22.6.R325.93","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1200690"},{"key":"2015110216263202000_22.6.R325.94","doi-asserted-by":"publisher","DOI":"10.1016\/0022-4731(78)90015-8"},{"key":"2015110216263202000_22.6.R325.95","doi-asserted-by":"crossref","unstructured":"Tran TB Liou D Menon VG Nissen NN 2013 Surgical management of advanced adrenocortical carcinoma: a 21-year population-based analysis. American Surgeon 79 1115\u20131118.","DOI":"10.1177\/000313481307901033"},{"key":"2015110216263202000_22.6.R325.96","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2013.229"},{"key":"2015110216263202000_22.6.R325.97","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.11084"},{"key":"2015110216263202000_22.6.R325.98","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-09-0804"},{"key":"2015110216263202000_22.6.R325.99","doi-asserted-by":"publisher","DOI":"10.1210\/en.2009-1404"}],"container-title":["Endocrine-Related Cancer"],"original-title":[],"link":[{"URL":"https:\/\/erc.bioscientifica.com\/view\/journals\/erc\/22\/6\/R325.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/erc.bioscientifica.com\/downloadpdf\/journals\/erc\/22\/6\/R325.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:53:16Z","timestamp":1784555596000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/erc\/article\/22\/6\/R325-R338\/33948"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,8,10]]},"references-count":99,"journal-issue":{"issue":"6"},"URL":"https:\/\/doi.org\/10.1530\/erc-15-0318","relation":{},"ISSN":["1351-0088","1479-6821"],"issn-type":[{"value":"1351-0088","type":"print"},{"value":"1479-6821","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,8,10]]}}}