小儿肾上腺皮质癌（pACC）的组织病理学分化很困难，且尚未评估临床预测和分层评分。因此，本综述旨在总结当前关于两个常用评分系统（Weiss/AFIP）评价pACC价值和准确性的证据。基于此，我们可以评估患者是否可以从唯一评分系统中获益。为此，我们对已发表的文献进行了系统回顾，并在我们的分析中包括了128名患者。大多数（72％）pACC具有良好的临床病程。随访时间范围为0-420个月，诊断时的平均年龄为5.6岁。临床病程良好的患者年龄较小（平均为4.8岁），而且不良预后的患者年龄较大（平均为7.6岁）。比较两个评分系统，Weiss评分的特异度非常低（25％），而灵敏度为100％。根据AFIP评分，特异度（77％）比Weiss评分高，而AFIP评分的灵敏度略低，为92％。随着年龄的增长，可以看出年龄差异，4岁以下婴儿的特异度比老年儿童（32％）低（20％）。相反，4岁以下婴儿的AFIP评分特异度比老年人群（76％）高（82％）。总结我们的结果，我们可以展示Weiss评分与AFIP评分相比不适合于预测pACC的恶性度，但进一步努力可以通过增强评分确保早期和准确的分层。

Histopathological differentiation in pediatric adrenocortical carcinoma (pACC) is difficult and clinical prediction and stratification scores are not evaluated yet. Therefore, this review aims to summarize current evidence on the value and accuracy of the two commonly used scoring systems (Weiss/AFIP) pACC. On this base, one might be able to evaluate if patients may benefit from a unique scoring system. For this we performed a systematic review of the published literature and included 128 patients in our analysis. The majority (72%) of the pACCs had a good clinical course. The follow up time ranged from 0-420 months with a mean age of 5.6 years at diagnosis. Patients with a good clinical course were younger (mean 4.8y) than patients with a poor outcome (mean 7.6y). Comparing the two scoring systems, the specificity of the Weiss score was very low (25%), whereas the sensitivity was 100%. According to the AFIP score, specificity (77%) was higher than Weiss score, whereas the sensitivity of the AFIP score was minimal lower with 92%. Age differences were recognizable as the specificity was lower in infants <4y (20%) than in older children (32%). In contrast, the specificity of the AFIP score was higher in infants <4 y (82%) than in older age groups (76%). Summarizing our results we could show, that the Weiss score is not a suitable tool for the prediction of malignancy in pACC in comparison with the AFIP score, but further efforts may seek to ensure early and accurate stratification through augmented scoring.