{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T08:36:52Z","timestamp":1777106212048,"version":"3.51.4"},"reference-count":20,"publisher":"Journal of the ASEAN Federation of Endocrine Societies (JAFES)","license":[{"start":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T00:00:00Z","timestamp":1776643200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J ASEAN Fed Endocr Soc"],"abstract":"<jats:p>Adrenocortical carcinomas are among the rarest and most aggressive paediatric endocrine neoplasms. We report a case of a functional adrenocortical carcinoma in a 6-year and 7-month-old female who initially presented with hypertensive encephalopathy, later progressing to overt Cushing syndrome with refractory hypertension. Given the presence of distant metastases, the patient was commenced on neoadjuvant chemotherapy and mitotane. Genetic testing for TP53 to evaluate Li-Fraumeni syndrome was declined. Despite initial response to the treatment, the disease remained refractory, and the patient succumbed after seven months of therapy.<\/jats:p>","DOI":"10.15605\/jafes.041.01.5167","type":"journal-article","created":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T07:49:25Z","timestamp":1777103365000},"source":"Crossref","is-referenced-by-count":0,"title":["A Rare Paediatric Adrenocortical Carcinoma with Aggressive Clinical Course"],"prefix":"10.15605","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-3430-7397","authenticated-orcid":false,"given":"Kah Yin","family":"Lim","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4607-3662","authenticated-orcid":false,"given":"Pian Pian","family":"Tee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"6244","published-online":{"date-parts":[[2026,4,20]]},"reference":[{"key":"84346","unstructured":"NCCR*Explorer Application. In: Incidence of Adrenocortical Tumors. 2024. National Cancer Institute: NCCR Cancer Statistics."},{"key":"84347","doi-asserted-by":"crossref","unstructured":"Michalkiewicz E, Sandrini R, Figueiredo B, et al. Clinical and outcome characteristics of children with adrenocortical tumors: A report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol. 2004;22(5):838-45 https:\/\/pubmed.ncbi.nlm.nih.gov\/14990639 https:\/\/doi.org\/10.1200\/JCO.2004.08.085","DOI":"10.1200\/JCO.2004.08.085"},{"key":"84348","doi-asserted-by":"crossref","unstructured":"Ribeiro RC, Figueiredo B. Childhood adrenocortical tumours. Eur J Cancer. 2004;40(8):1117-26. https:\/\/pubmed.ncbi.nlm.nih.gov\/15110875 https:\/\/doi.org\/10.1016\/j.ejca.2004.01.031","DOI":"10.1016\/j.ejca.2004.01.031"},{"key":"84349","doi-asserted-by":"crossref","unstructured":"Wasserman JD, Novokmet A, Eichler-Jonsson C, et al. Prevalence and functional consequence of TP53 mutations in pediatric adrenocortical carcinoma: A children's oncology group study. J Clin Oncol. 2015;33(6):602-9. https:\/\/pubmed.ncbi.nlm.nih.gov\/25584008 https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC4517369 https:\/\/doi.org\/10.1200\/JCO.2013.52.6863","DOI":"10.1200\/JCO.2013.52.6863"},{"key":"84350","doi-asserted-by":"crossref","unstructured":"Cust\u00f3dio G, Parise GA, Kiesel Filho N, et al. Impact of neonatal screening and surveillance for the TP53 R337H mutation on early detection of childhood adrenocortical tumors. J Clin Oncol. 2013;31(20):2619-26. https:\/\/pubmed.ncbi.nlm.nih.gov\/23733769","DOI":"10.1200\/JCO.2012.46.3711"},{"key":"84351","unstructured":"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC3808236"},{"key":"84352","doi-asserted-by":"crossref","unstructured":"https:\/\/doi.org\/10.1200\/JCO.2012.46.3711","DOI":"10.1200\/JCO.2012.46.3711"},{"key":"84353","doi-asserted-by":"crossref","unstructured":"Costa TEJ, Gerber VKQ, Iba\u00f1ez HC, et al. Penetrance of the TP53 R337H Mutation and Pediatric Adrenocortical Carcinoma Incidence Associated with Environmental Influences in a 12-Year Observational Cohort in Southern Brazil. Cancers (Basel). 2019;11(11):1804. https:\/\/pubmed.ncbi.nlm.nih.gov\/31744167 https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC6896071 https:\/\/doi.org\/10.3390\/cancers11111804","DOI":"10.3390\/cancers11111804"},{"key":"84354","doi-asserted-by":"crossref","unstructured":"Cecchetto G, Ganarin A, Bien E, et al. Outcome and prognostic factors in high-risk childhood adrenocortical carcinomas: A report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Pediatr Blood Cancer. 2017;64(6). https:\/\/pubmed.ncbi.nlm.nih.gov\/27957799 https:\/\/doi.org\/10.1002\/pbc.26368","DOI":"10.1002\/pbc.26368"},{"key":"84355","doi-asserted-by":"crossref","unstructured":"Rodriguez-Galindo C, Krailo MD, Pinto EM, et al. Treatment of pediatric adrenocortical carcinoma with surgery, retroperitoneal lymph node dissection, and chemotherapy: The Children's Oncology Group ARAR0332 protocol. J Clin Oncol. 2021;39(22):2463-73. https:\/\/pubmed.ncbi.nlm.nih.gov\/33822640 https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC8462560","DOI":"10.1200\/JCO.20.02871"},{"key":"84356","doi-asserted-by":"crossref","unstructured":"https:\/\/doi.org\/10.1200\/JCO.20.02871","DOI":"10.1200\/JCO.20.02871"},{"key":"84357","doi-asserted-by":"crossref","unstructured":"Gupta N, Rivera M, Novotny P, Rodriguez V, Bancos I, Lteif A. Adrenocortical carcinoma in children: A clinicopathological analysis of 41 patients at the Mayo Clinic from 1950 to 2017. Horm Res Paediatr. 2018;90(1):8-18. https:\/\/pubmed.ncbi.nlm.nih.gov\/29804118","DOI":"10.1159\/000488855"},{"key":"84358","doi-asserted-by":"crossref","unstructured":"https:\/\/doi.org\/10.1159\/000488855","DOI":"10.1159\/000488855"},{"key":"84359","doi-asserted-by":"crossref","unstructured":"Street ME, Weber A, Camacho-H\u00fcbner C, Perry LA, Brain CE, Cotterill AM, Savage MO. Girls with virilisation in childhood: A diagnostic protocol for investigation. J Clin Pathol. 1997;50(5):379-83. https:\/\/pubmed.ncbi.nlm.nih.gov\/9215119","DOI":"10.1136\/jcp.50.5.379"},{"key":"84360","doi-asserted-by":"crossref","unstructured":"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC499938 https:\/\/doi.org\/10.1136\/jcp.50.5.379","DOI":"10.1136\/jcp.50.5.379"},{"key":"84361","doi-asserted-by":"crossref","unstructured":"Wieneke JA, Thompson LD, Heffess CS. Adrenal cortical neoplasms in the pediatric population: A clinicopathologic and immunophenotypic analysis of 83 patients. Am J Surg Pathol. 2003;27(7):867-81. https:\/\/pubmed.ncbi.nlm.nih.gov\/12826878","DOI":"10.1097\/00000478-200307000-00001"},{"key":"84362","doi-asserted-by":"crossref","unstructured":"https:\/\/doi.org\/10.1097\/00000478-200307000-00001","DOI":"10.1097\/00000478-200307000-00001"},{"key":"84363","doi-asserted-by":"crossref","unstructured":"Castinetti F, Guignat L, Giraud P, et al. Ketoconazole in Cushing's disease: Is it worth a try? J Clin Endocrinol Metab. 2014;99(5):1623-30. https:\/\/pubmed.ncbi.nlm.nih.gov\/24471573 https:\/\/doi.org\/10.1210\/jc.2013-3628","DOI":"10.1210\/jc.2013-3628"},{"key":"84364","doi-asserted-by":"crossref","unstructured":"Le Bihan C, Moutou C, Brugi\u00e8res L, Feunteun J, Bona\u00efti-Pelli\u00e9 C. ARCAD: A method for estimating age-dependent disease risk associated with mutation carrier status from family data. 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