{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T04:24:32Z","timestamp":1790915072712,"version":"4.1.0"},"reference-count":35,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"published-print":{"date-parts":[[2018]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background\/Aims:&lt;\/i&gt;&lt;\/b&gt; Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms \u226421 years). Outcomes of interest were: alive with no evidence of disease, alive with evidence of disease, and dead of disease. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Median age at onset of symptoms was 15.7 years (&lt;i&gt;n&lt;\/i&gt; = 41; range, 0.2\u201321 years). Female:male ratio was 3.6: 1. Mixed symptomatology (&amp;#x3e; 1 hormone abnormality) was the most common presentation (54%, &lt;i&gt;n&lt;\/i&gt; = 22). Sixty-six percent of patients (&lt;i&gt;n&lt;\/i&gt; = 27 out of 41) underwent total adrenalectomy. Metastatic disease was more common in children aged &amp;#x3e; 12 years (&lt;i&gt;p&lt;\/i&gt; = 0.002 compared to &amp;#x3c; 4 years). The most common sites of metastases were the liver and lungs. Overall 2-year and 5-year survival rates were 61% (95% CI 45\u201377) and 46% (95% CI 30\u201362), respectively. Metastasis at the time of diagnosis was independently associated with poor prognosis (risk ratio 13.7%; 95% CI 3.9\u201387.7). Weiss criteria (29%) and modified Weiss criteria (33%) were less accurate in younger patients (&amp;#x3c; 12 years), compared to the Wieneke index (100%). &lt;b&gt;&lt;i&gt;Conclusion:&lt;\/i&gt;&lt;\/b&gt; The presence of metastases was an independent prognostic factor. The Wieneke index was the most accurate in predicting clinical outcomes in younger children.<\/jats:p>","DOI":"10.1159\/000488855","type":"journal-article","created":{"date-parts":[[2018,5,25]],"date-time":"2018-05-25T21:01:19Z","timestamp":1527282079000},"page":"8-18","source":"Crossref","is-referenced-by-count":69,"title":["Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 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