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• W2013170178 abstract "Abstract Childhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low. Little is known about their pathogenesis, clinical presentation, and optimal treatment. In recent years, however, new information has been derived from the International Pediatric Adrenocortical Tumor Registry (IPACTR), and new clues to its pathogenesis have emerged. To provide an overview of the available data that may apply to pediatric ACT, we reviewed the epidemiology, pathogenesis, and treatment of ACT in adults and in children. Germline TP53 mutation is almost always the predisposing factor in childhood ACT. A unique germline mutation ( TP53 – R337H ) has been described in Southern Brazil, where the incidence of ACT is 10–15 times the general incidence. Childhood ACT typically present during the first 5 years of life and has female predominance. Hormone hyperproduction is almost universal, and most patients present with virilization. Two‐thirds of patients have resectable tumors. Surgery is the definitive treatment for ACT, and a curative complete resection should always be attempted. Cisplatin‐based chemotherapy with mitotane is indicated for unresectable or metastatic disease, although its impact on overall outcome is slight. In childhood ACT, age, tumor size, and tumor resectability are the most important prognostic indicators. Outcome is stage‐dependent; patients with small, resectable tumors have survival rates in excess of 80%, whereas the outcome for patients with unresectable disease is dismal. Patients with large, resectable tumors have an intermediate outcome. Childhood ACT are rare, but their unique epidemiology appear to implicate novel oncogenic pathways that are unique to the pediatric population. Multi‐institutional and prospective studies are necessary to further our understanding of the pathogenesis and to improve outcomes. © 2005 Wiley‐Liss, Inc." @default.
• W2013170178 created "2016-06-24" @default.
• W2013170178 creator A5030630722 @default.
• W2013170178 creator A5053244337 @default.
• W2013170178 creator A5075849954 @default.
• W2013170178 creator A5086250633 @default.
• W2013170178 date "2005-03-03" @default.
• W2013170178 modified "2023-10-17" @default.
• W2013170178 title "Biology, clinical characteristics, and management of adrenocortical tumors in children" @default.
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• W2013170178 doi "https://doi.org/10.1002/pbc.20318" @default.
• W2013170178 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15747338" @default.
• W2013170178 hasPublicationYear "2005" @default.
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