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- W4211058838 abstract "Childhood adrenocortical tumor (ACT) represents only about 0.2% of all pediatric malignancies. At the time of diagnosis, most children show signs and symptoms of virilization. Fewer than 10% of patients with ACT show no endocrine syndrome at presentation. ACT is commonly associated with constitutional genetic abnormalities, particularly mutations of the TP53 gene and constitutional abnormalities of chromosome 11p15.5 (Beckwith–Wiedemann syndrome). A unique germline TP53 mutation (R337H) has been described in southern Brazil, where the incidence of ACT is about 10 times that in other geographic regions. Histological features are used to classify ACTs as adenomas or carcinomas. The extent of disease is best evaluated by computed tomography or magnetic resonance imaging; the role of positron emission tomography scans is still evolving. Complete tumor resection is required to cure ACT. The role of chemotherapy has not been established, although definitive responses to several anticancer drugs have been documented. Among patients who undergo complete tumor resection, prognostic factors include the age, tumor size, endocrine syndrome at presentation, gene expression, and tumor histology. Some children with ACT show abnormalities of growth and development at the time of presentation, but these usually resolve after surgery." @default.
- W4211058838 created "2022-02-13" @default.
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- W4211058838 creator A5075849954 @default.
- W4211058838 creator A5086250633 @default.
- W4211058838 date "2017-03-11" @default.
- W4211058838 modified "2023-10-17" @default.
- W4211058838 title "Uncommon Adrenal Tumors in Children and Adolescents" @default.
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