Adrenocortical tumor (ACT) is a rare disease with an annual worldwide incidence of 0.3–0.38/million children below 15 years old, and Brazilian population presents the highest incidence because of germline mutation in the TP53. Pediatric ACT is associated with virilizing features and hypercortisolism in most cases. Malignancy is defined when local invasion or metastasis is found, and it is associated with a poor prognosis. However, the correct and early diagnosis and treatment may impact on overall and disease-free survival.

中文翻译：

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小儿肾上腺皮质肿瘤-审查和管理更新。

肾上腺皮质肿瘤（ACT）是一种罕见的疾病，全世界每年的发病率为0.3–0.38 /每15岁以下的百万儿童，由于TP53中的种系突变，巴西人群的发病率最高。在大多数情况下，儿科ACT与男性化特征和皮质醇过多有关。恶性是在发现局部浸润或转移时定义的，并与不良预后相关。但是，正确的早期诊断和治疗可能会影响总体生存率和无病生存期。