{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T04:24:33Z","timestamp":1790915073853,"version":"4.1.0"},"reference-count":88,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2020,5,1]],"date-time":"2020-05-01T00:00:00Z","timestamp":1588291200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,5,1]],"date-time":"2020-05-01T00:00:00Z","timestamp":1588291200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100012524","name":"American Lebanese Syrian Associated Charities","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012524","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Best Practice & Research Clinical Endocrinology & Metabolism"],"published-print":{"date-parts":[[2020,5]]},"DOI":"10.1016\/j.beem.2020.101448","type":"journal-article","created":{"date-parts":[[2020,7,3]],"date-time":"2020-07-03T12:16:31Z","timestamp":1593778591000},"page":"101448","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":57,"title":["Pediatric adrenocortical tumours"],"prefix":"10.1016","volume":"34","author":[{"given":"Emilia Modolo","family":"Pinto","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerard P.","family":"Zambetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Rodriguez-Galindo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.beem.2020.101448_bib1","series-title":"Cancer incidence and survival among children and adolescents: United States SEER program 1975-1995","first-page":"139","article-title":"Carcinomas and other malignant epithelial neoplasms","author":"Bernstein","year":"1999"},{"key":"10.1016\/j.beem.2020.101448_bib2","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1002\/pbc.20318","article-title":"Biology, clinical characteristics, and management of adrenocortical tumors in children","volume":"45","author":"Rodriguez-Galindo","year":"2005","journal-title":"Pediatr Blood Canc"},{"key":"10.1016\/j.beem.2020.101448_bib3","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1200\/JCO.2004.08.085","article-title":"Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry","volume":"22","author":"Michalkiewicz","year":"2004","journal-title":"J\u00a0Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib4","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.mce.2011.10.015","article-title":"The International Pediatric Adrenocortical Tumor Registry initiative: contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors","volume":"351","author":"Ribeiro","year":"2012","journal-title":"Mol Cell Endocrinol"},{"key":"10.1016\/j.beem.2020.101448_bib5","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.ecl.2015.02.001","article-title":"Development of adrenal cortex zonation","volume":"44","author":"Xing","year":"2015","journal-title":"Endocrinol Metab Clin N Am"},{"key":"10.1016\/j.beem.2020.101448_bib6","doi-asserted-by":"crossref","first-page":"6302","DOI":"10.1038\/ncomms7302","article-title":"Genomic landscape of paediatric adrenocortical tumours","volume":"6","author":"Pinto","year":"2015","journal-title":"Nat Commun"},{"key":"10.1016\/j.beem.2020.101448_bib7","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1038\/ng.2953","article-title":"Integrated genomic characterization of adrenocortical carcinoma","volume":"46","author":"Assie\u00b4","year":"2014","journal-title":"Nat Genet"},{"key":"10.1016\/j.beem.2020.101448_bib8","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/j.ccell.2016.04.002","article-title":"Comprehensive pan-genomic characterization of adrenocortical carcinoma","volume":"29","author":"Zheng","year":"2016","journal-title":"Canc Cell"},{"key":"10.1016\/j.beem.2020.101448_bib9","series-title":"Associate professor of surgery, the Johns Hopkins university, &c pp. 341.319 illustrations","article-title":"The pituitary body and its disorders: clinical states produced by disorders of the hypophysis cerebri","author":"McKenzie","year":"1913"},{"key":"10.1016\/j.beem.2020.101448_bib10","series-title":"The history of clinical endocrinology: a comprehensive account of endocrinology from earliest times to the present day","author":"Medvei","year":"1982"},{"key":"10.1016\/j.beem.2020.101448_bib11","first-page":"II40","article-title":"Adenoma of the adrenal cortex: with report of a case","volume":"I99","author":"Hicks","year":"1928","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.beem.2020.101448_bib12","first-page":"204","article-title":"Genito-suprarenal syndrome (suprarenal virilism) in a girl one and a half years old, with successful operation","volume":"27","author":"Collett","year":"1924","journal-title":"Am J Dis Child"},{"key":"10.1016\/j.beem.2020.101448_bib13","first-page":"189","article-title":"On the relation of the suprarenal capsules to the sexual organs","volume":"56","author":"Bulloch","year":"1905","journal-title":"Trans Jpn Pathol Soc Lond"},{"key":"10.1016\/j.beem.2020.101448_bib14","first-page":"501","article-title":"Dystrophies en relation avec des lesions de capsules surre'nales, hirsutisme et progeria","volume":"12","author":"Apert","year":"1910","journal-title":"Bull Soc Pediatr Paris"},{"key":"10.1016\/j.beem.2020.101448_bib15","volume":"no. 225","author":"Gallais","year":"1912"},{"key":"10.1016\/j.beem.2020.101448_bib16","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1016\/j.jpedsurg.2013.02.017","article-title":"Predictors of survival in pediatric adrenocortical carcinoma: a surveillance, epidemiology, and end results (SEER) program study","volume":"48","author":"McAteer","year":"2013","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.beem.2020.101448_bib17","first-page":"305","article-title":"International variations in the incidence of childhood carcinomas","volume":"3","author":"Stiller","year":"1994","journal-title":"Canc Epidemiol Biomarkers Prev"},{"key":"10.1016\/j.beem.2020.101448_bib18","first-page":"1231","article-title":"Survey of cancer in children admitted to a Brazilian charity hospital","volume":"43","author":"Marigo","year":"1968","journal-title":"J\u00a0Natl Cancer Inst"},{"key":"10.1016\/j.beem.2020.101448_bib19","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1590\/S0004-27302004000500009","article-title":"Founder effect for the highly prevalent R337H mutation of tumor suppressor p53 in Brazilian patients with adrenocortical tumors","volume":"48","author":"Pinto","year":"2004","journal-title":"Arq Bras Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib20","doi-asserted-by":"crossref","first-page":"9330","DOI":"10.1073\/pnas.161479898","article-title":"An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma","volume":"98","author":"Ribeiro","year":"2001","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.beem.2020.101448_bib21","doi-asserted-by":"crossref","first-page":"4970","DOI":"10.1210\/jcem.86.10.7957","article-title":"An inherited mutation outside the highly conserved DNA-binding domain of the p53 tumor suppressor protein in children and adults with sporadic adrenocortical tumors","volume":"86","author":"Latronico","year":"2001","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib22","first-page":"2027","article-title":"Childhood adrenocortical tumors","volume":"82","author":"Sandrini","year":"1997","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib23","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.1093\/jnci\/86.22.1707","article-title":"High frequency of germline p53 mutations in childhood adrenocortical cancer","volume":"86","author":"Wagner","year":"1994","journal-title":"J\u00a0Natl Cancer Inst"},{"key":"10.1016\/j.beem.2020.101448_bib24","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1086\/302575","article-title":"Are there low-penetrance TP53 alleles? Evidence from childhood adrenocortical tumors","volume":"65","author":"Varley","year":"1999","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.beem.2020.101448_bib25","first-page":"1932","article-title":"P53 germline mutations in childhood cancers and cancer risk for carrier individuals","volume":"82","author":"Chompret","year":"2000","journal-title":"Br J Canc"},{"key":"10.1016\/j.beem.2020.101448_bib26","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1200\/JCO.2013.52.6863","article-title":"Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: a Children's Oncology Group study","volume":"20","author":"Wasserman","year":"2015","journal-title":"J\u00a0Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib27","doi-asserted-by":"crossref","first-page":"3956","DOI":"10.1200\/JCO.2017.74.2460","article-title":"Identification of clinical and biologic correlates associated with outcome in children with adrenocortical tumors without germline TP53 mutations: a St. Jude Adrenocortical Tumor Registry and Children's Oncology Group study","volume":"35","author":"Pinto","year":"2017","journal-title":"J\u00a0Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib28","series-title":"Adrenal and endocrine tumors in children","first-page":"265","article-title":"Adrenal cortical carcinoma in children: 42 patients treated from 1958 to 1980 at Villejuif","author":"Lefevre","year":"1983"},{"key":"10.1016\/j.beem.2020.101448_bib29","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1053\/jpsu.2001.22280","article-title":"Adrenocortical tumors in children","volume":"36","author":"Ciftci","year":"2001","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.beem.2020.101448_bib30","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1159\/000488855","article-title":"Adrenocortical carcinoma in children: a clinicopathological analysis of 41 patients at the Mayo Clinic from 1950 to 2017","volume":"90","author":"Gupta","year":"2018","journal-title":"Hormone Res Pediatr"},{"key":"10.1016\/j.beem.2020.101448_bib31","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1097\/00000478-198903000-00004","article-title":"Pathologic features of prognostic significance in adrenocortical carcinoma","volume":"13","author":"Weiss","year":"1989","journal-title":"Am J Surg Pathol"},{"key":"10.1016\/j.beem.2020.101448_bib32","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1097\/00000478-200307000-00001","article-title":"Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients","volume":"27","author":"Wieneke","year":"2003","journal-title":"Am J Surg Pathol"},{"key":"10.1016\/j.beem.2020.101448_bib33","doi-asserted-by":"crossref","first-page":"2027","DOI":"10.1210\/jc.2005-2639","article-title":"Clinical review: adrenocortical carcinoma: clinical update","volume":"91","author":"Allolio","year":"2006","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib34","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1016\/j.ejca.2004.01.031","article-title":"Childhood adrenocortical tumours","volume":"40","author":"Ribeiro","year":"2004","journal-title":"Eur J Canc"},{"key":"10.1016\/j.beem.2020.101448_bib35","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1159\/000134829","article-title":"Cushing syndrome caused by adrenocortical tumors and hyperplasias (corticotrophin-independent Cushing syndrome)","volume":"13","author":"Stratakis","year":"2008","journal-title":"Endocr Dev"},{"key":"10.1016\/j.beem.2020.101448_bib36","doi-asserted-by":"crossref","first-page":"45","DOI":"10.3389\/fcell.2015.00045","article-title":"Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment","volume":"3","author":"Libe","year":"2015","journal-title":"Front Cell Dev Biol"},{"key":"10.1016\/j.beem.2020.101448_bib37","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/S0094-0143(05)70089-6","article-title":"Adrenocortical carcinoma in children","volume":"27","author":"Liou","year":"2000","journal-title":"Urol Clin"},{"key":"10.1016\/j.beem.2020.101448_bib38","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1016\/j.surg.2003.07.013","article-title":"Intracaval endovascular ultrasonography for large adrenal and retroperitoneal tumors","volume":"134","author":"Kikumori","year":"2003","journal-title":"Surgery"},{"key":"10.1016\/j.beem.2020.101448_bib39","doi-asserted-by":"crossref","first-page":"2358","DOI":"10.1210\/jc.2008-0050","article-title":"[123 I] Iodometomidate for molecular imaging of adrenocortical cytochrome P450 family 11B enzymes","volume":"93","author":"Hahner","year":"2008","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib40","doi-asserted-by":"crossref","first-page":"94","DOI":"10.3109\/00313029109060804","article-title":"Adrenal cortical tumors in childhood-clinicopathological features of six cases","volume":"23","author":"Moore","year":"1991","journal-title":"Pathology"},{"key":"10.1016\/j.beem.2020.101448_bib41","doi-asserted-by":"crossref","first-page":"1","DOI":"10.5114\/aoms.2016.57577","article-title":"Chromogranin A \u2013 unspecific neuroendocrine marker. Clinical utility and potential diagnostic pitfalls","volume":"12","author":"Gut","year":"2016","journal-title":"Arch Med Sci"},{"key":"10.1016\/j.beem.2020.101448_bib42","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1530\/eje.0.1450335","article-title":"Discerning malignancy in adrenocortical tumors: are molecular markers useful","volume":"145","author":"Wachenfeld","year":"2001","journal-title":"Eur J Endocrinol"},{"key":"10.1016\/j.beem.2020.101448_bib43","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1111\/his.13255","article-title":"Challenges in surgical pathology of adrenocortical tumors","volume":"72","author":"Erickson","year":"2018","journal-title":"Histopathology"},{"key":"10.1016\/j.beem.2020.101448_bib44","doi-asserted-by":"crossref","first-page":"e401","DOI":"10.1200\/JCO.2009.27.5958","article-title":"Adjuvant therapy in patients with adrenocortical carcinoma: a position of an international panel","volume":"28","author":"Berruti","year":"2010","journal-title":"J\u00a0Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib45","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1210\/jc.2014-3182","article-title":"Major prognostic role of Ki67 in localized adrenocortical carcinoma after complete resection","volume":"100","author":"Beuschlein","year":"2015","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib46","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1007\/s00423-010-0698-y","article-title":"Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade","volume":"395","author":"Miller","year":"2015","journal-title":"Langenbeck's Arch Surg"},{"key":"10.1016\/j.beem.2020.101448_bib47","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1158\/0008-5472.CAN-06-3767","article-title":"Gene expression profiling of childhood adrenocortical tumors","volume":"67","author":"West","year":"2007","journal-title":"Canc Res"},{"key":"10.1016\/j.beem.2020.101448_bib48","doi-asserted-by":"crossref","first-page":"6247","DOI":"10.1158\/1078-0432.CCR-15-2738","article-title":"Prognostic significance of major histocompatibility complex class II expression in pediatric adrenocortical tumors: a St. Jude and Children's Oncology Group study","volume":"22","author":"Pinto","year":"2016","journal-title":"Clin Canc Res"},{"key":"10.1016\/j.beem.2020.101448_bib49","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1007\/s00268-010-0532-2","article-title":"Laparoscopic resection is inappropriate in patients with known or suspected adrenocortical carcinoma","volume":"34","author":"Miller","year":"2010","journal-title":"World J Surg"},{"key":"10.1016\/j.beem.2020.101448_bib50","series-title":"ARAR0332. Treatment of adrenocortical tumors with surgery plus lymph node dissection and multiagent chemotherapy: a groupwide phase III study","author":"Children's Oncology Group","year":"2010"},{"key":"10.1016\/j.beem.2020.101448_bib51","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1007\/s002689900442","article-title":"Adrenocortical carcinoma: surgery and mitotane for treatment and steroid profiles for follow-up","volume":"22","author":"Khorram-Manesh","year":"1998","journal-title":"World J Surg"},{"key":"10.1016\/j.beem.2020.101448_bib52","doi-asserted-by":"crossref","first-page":"2372","DOI":"10.1056\/NEJMoa063360","article-title":"Adjuvant mitotane treatment for adrenocortical carcinoma","volume":"356","author":"Terzolo","year":"2007","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.beem.2020.101448_bib53","first-page":"62","article-title":"Role of mitotane in adrenocortical carcinoma \u2013 review and state of the art","volume":"14","author":"Paragliola","year":"2018","journal-title":"Eur Endocrinol"},{"key":"10.1016\/j.beem.2020.101448_bib54","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1055\/s-0032-1327579","article-title":"Systemic treatment of adrenocortical carcinoma in children: data from the German GPOH-MET 97 trial","volume":"224","author":"Redlich","year":"2012","journal-title":"Klin P\u00e4diatr"},{"key":"10.1016\/j.beem.2020.101448_bib55","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1097\/01.mph.0000212965.52759.1c","article-title":"Mitotane associated with cisplatin, etoposide, and doxorubicin in advanced childhood adrenocortical carcinoma: mitotane monitoring and tumor regression","volume":"28","author":"Zancanella","year":"2006","journal-title":"J\u00a0Pediatr Hematol Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib56","doi-asserted-by":"crossref","first-page":"1740","DOI":"10.1158\/1078-0432.CCR-12-3354","article-title":"Establishment and characterization of the first pediatric adrenocortical carcinoma xenograft model identifies topotecan as a potential chemotherapeutic agent","volume":"19","author":"Pinto","year":"2013","journal-title":"Clin Canc Res"},{"key":"10.1016\/j.beem.2020.101448_bib57","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.coemr.2019.08.009","article-title":"Contemporary preclinical human models of adrenocortical carcinoma","volume":"8","author":"Pinto","year":"2019","journal-title":"Curr Opin Endocr Metab Res"},{"key":"10.1016\/j.beem.2020.101448_bib58","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/j.jpedsurg.2004.01.029","article-title":"A\u00a0surgical approach to adrenocortical tumors in children: the mainstay of treatment","volume":"39","author":"Stewart","year":"2004","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.beem.2020.101448_bib59","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1002\/cncr.24030","article-title":"Limited prognostic value of the 2004 International Union against Cancer staging classification for adrenocortical carcinoma: proposal for a revised TNM classification","volume":"115","author":"Fassnacht","year":"2009","journal-title":"Cancer"},{"issue":"6","key":"10.1016\/j.beem.2020.101448_bib60","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1016\/j.jpedsurg.2011.03.052","article-title":"Adrenal cortical tumors in children: factors associated with poor outcome","volume":"46","author":"Klein","year":"2011","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.beem.2020.101448_bib61","article-title":"Outcome and prognostic factors in high-risk childhood adrenocortical carcinomas: a report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)","volume":"64","author":"Cecchetto","year":"2016","journal-title":"Pediatr Blood Canc"},{"key":"10.1016\/j.beem.2020.101448_bib62","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.jpedsurg.2015.10.039","article-title":"Factors associated with survival in pediatric adrenocortical carcinoma: an analysis of the National Cancer Data Base (NCDB)","volume":"51","author":"Gulack","year":"2016","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.beem.2020.101448_bib63","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.humpath.2011.04.016","volume":"43","author":"Magro","year":"2012","journal-title":"Hum Pathol"},{"key":"10.1016\/j.beem.2020.101448_bib64","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1016\/0092-8674(94)90211-9","article-title":"A\u00a0cell-specific nuclear receptor is essential for adrenal and gonadal development and sexual differentiation","volume":"20","author":"Luo","year":"1994","journal-title":"Cell"},{"key":"10.1016\/j.beem.2020.101448_bib65","first-page":"378","article-title":"Developmental and functional biology of the primate fetal adrenal cortex","volume":"18","author":"Mesiano","year":"1997","journal-title":"Endocr Rev"},{"key":"10.1016\/j.beem.2020.101448_bib66","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1016\/j.crad.2005.04.006","article-title":"Embryology of the adrenal glands and its relevance to diagnostic imaging","volume":"60","author":"Barwick","year":"2005","journal-title":"Clin Radiol"},{"issue":"23","key":"10.1016\/j.beem.2020.101448_bib67","article-title":"Pediatric adrenocortical tumors: what they can tell us on adrenal development and comparison with adult adrenal tumors","volume":"6","author":"Lalli","year":"2015","journal-title":"Front Endocrinol"},{"key":"10.1016\/j.beem.2020.101448_bib68","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1038\/nrclinonc.2014.41","article-title":"Li-Fraumeni syndrome: cancer risk assessment and clinical management","volume":"11","author":"McBride","year":"2014","journal-title":"Nat Rev Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib69","first-page":"1365","article-title":"Rhabdomyosarcoma in children: epidemiologic study and identification of a familial cancer syndrome","volume":"43","author":"Li","year":"1969","journal-title":"J\u00a0Natl Cancer Inst"},{"key":"10.1016\/j.beem.2020.101448_bib70","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1126\/science.1978757","article-title":"Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms","volume":"250","author":"Malkin","year":"1990","journal-title":"Science"},{"key":"10.1016\/j.beem.2020.101448_bib71","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1093\/jnci\/90.8.606","article-title":"Multiple primary cancers in families with Li-Fraumeni syndrome","volume":"90","author":"Hisada","year":"1998","journal-title":"J\u00a0Natl Cancer Inst"},{"key":"10.1016\/j.beem.2020.101448_bib72","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1038\/35042675","article-title":"Surfing the p53 network","volume":"408","author":"Vogelstein","year":"2000","journal-title":"Nature"},{"key":"10.1016\/j.beem.2020.101448_bib73","first-page":"978312","article-title":"Recent advances in p53 research and cancer treatment","volume":"2011","author":"Suzuki","year":"2011","journal-title":"J\u00a0Biomed Biotechnol"},{"key":"10.1016\/j.beem.2020.101448_bib74","doi-asserted-by":"crossref","first-page":"E476","DOI":"10.1210\/jc.2011-1982","article-title":"TP53 germline mutations in adult patients with adrenocortical carcinoma","volume":"97","author":"Herrmann","year":"2012","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib75","doi-asserted-by":"crossref","first-page":"E119","DOI":"10.1210\/jc.2012-2198","article-title":"Prevalence of germline TP53 mutations in a prospective series of unselected patients with adrenocortical carcinoma","volume":"98","author":"Raymond","year":"2013","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib76","doi-asserted-by":"crossref","first-page":"2619","DOI":"10.1200\/JCO.2012.46.3711","article-title":"Impact of neonatal screening and surveillance for the TP53 R337H mutation on early detection of childhood adrenocortical tumors","volume":"31","author":"Cust\u00f3dio","year":"2013","journal-title":"J\u00a0Clin Oncol"},{"key":"10.1016\/j.beem.2020.101448_bib77","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0018015","article-title":"Increased incidence of choroid plexus carcinoma due to the germline TP53 R337H mutation in southern Brazil","volume":"6","author":"Custodio","year":"2011","journal-title":"PloS One"},{"key":"10.1016\/j.beem.2020.101448_bib78","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1590\/S1807-59322007000200012","article-title":"Classical osteoblastoma, atypical osteoblastoma, and osteosarcoma: a comparative study based on clinical, histological, and biological parameters","volume":"62","author":"Oliveira","year":"2007","journal-title":"Clinics"},{"key":"10.1016\/j.beem.2020.101448_bib79","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1897-4287-12-8","article-title":"TP53 p.R337H prevalence in a series of Brazilian hereditary breast cancer families","volume":"12","author":"Cury","year":"2014","journal-title":"Hered Cancer Clin Pract"},{"key":"10.1016\/j.beem.2020.101448_bib80","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1136\/jmg.2009.066704","article-title":"TP53 PIN3 and MDM2 SNP309 polymorphisms as genetic modifiers in the Li-Fraumeni syndrome: impact on age at first diagnosis","volume":"46","author":"Marcel","year":"2009","journal-title":"J\u00a0Med Genet"},{"key":"10.1016\/j.beem.2020.101448_bib81","series-title":"Gene Reviews Seattle (WA)","article-title":"Beckwith-Wiedemann syndrome","author":"Shuman","year":"1993"},{"key":"10.1016\/j.beem.2020.101448_bib82","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1038\/ejhg.2009.106","article-title":"Beckwith-Wiedemann syndrome","volume":"18","author":"Weksberg","year":"2010","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.beem.2020.101448_bib83","first-page":"235","article-title":"Fetal adrenal development: insight gained from adrenal tumors","volume":"16","author":"Coulter","year":"2005"},{"key":"10.1016\/j.beem.2020.101448_bib84","first-page":"2559","article-title":"Structural and functional abnormalities at 11p15 are associated with the malignant phenotype in sporadic adrenocortical tumors: study on a series of 82 tumors","volume":"82","author":"Gicquel","year":"1997","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib85","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1158\/1078-0432.CCR-08-1067","article-title":"Molecular classification and prognostication of adrenocortical tumors by transcriptome profiling","volume":"15","author":"Giordano","year":"2009","journal-title":"Clin Canc Res"},{"key":"10.1016\/j.beem.2020.101448_bib86","doi-asserted-by":"crossref","first-page":"E174","DOI":"10.1210\/jc.2012-2993","article-title":"Identification of a CpG island methylator phenotype in adrenocortical carcinomas","volume":"98","author":"Barreau","year":"2013","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib87","first-page":"923","article-title":"DNA methylation is an independent prognostic marker of survival in adrenocortical cancer","volume":"102","author":"Jouinot","year":"2017","journal-title":"J\u00a0Clin Endocrinol Metabol"},{"key":"10.1016\/j.beem.2020.101448_bib88","doi-asserted-by":"crossref","DOI":"10.1200\/PO.19.00163","article-title":"DNA methylation profiling reveals prognostically significant groups in pediatric adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry","author":"Clay","year":"2019","journal-title":"JCO Precis Oncol"}],"container-title":["Best Practice & Research Clinical Endocrinology & Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521690X20300750?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521690X20300750?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T16:55:42Z","timestamp":1761497742000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1521690X20300750"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,5]]},"references-count":88,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2020,5]]}},"alternative-id":["S1521690X20300750"],"URL":"https:\/\/doi.org\/10.1016\/j.beem.2020.101448","relation":{},"ISSN":["1521-690X"],"issn-type":[{"value":"1521-690X","type":"print"}],"subject":[],"published":{"date-parts":[[2020,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pediatric adrenocortical tumours","name":"articletitle","label":"Article Title"},{"value":"Best Practice & Research Clinical Endocrinology & Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.beem.2020.101448","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"101448"}}