Abstract：
Adrenocortical neoplasms are rare in childhood. Their histopathological categorization into benign and malignant is often challenging, impacting further management. While the AFIP/Wieneke scoring system is widely used for the prognostic classification of these tumors, it has limitations. Few other tumor scoring systems have evolved over the past few years. These have been validated in adults but not yet in pediatric patients. We evaluated a cohort of pediatric adrenocortical neoplasms to assess the applicability of AFIP/Wieneke criteria and the recently introduced Helsinki score and reticulin algorithm in predicting clinical outcomes. A tumor was considered \'clinically aggressive\' in the presence of any of the following: metastases, recurrence, progressive disease, or death due to disease. Cases without any such event were considered \'clinically good\'. Event-free survival time was the duration from the date of clinical presentation to any post-operative adverse event. For overall survival analysis, the endpoint was either the last follow-up or death due to disease.Using ROC curve analysis, the obtained cut-off Helsinki score of 24 could stratify the cases into two prognostically relevant groups. Survival analysis showed significant differences in the event-free and overall survival of these two groups of patients, validating the proposed cut-off. None of the three histopathological scoring systems could predict an unfavorable outcome with 100% accuracy. All showed a sensitivity of ≥ 80%, with the reticulin algorithm achieving 100% sensitivity. The specificity and accuracy of the AFIP/Wieneke criteria were the lowest (62.5% and 73.08%, respectively). While the Helsinki score (at the cut-off score of 24) and the reticulin algorithm had similar accuracy rates (80.77%, and 80%, respectively), the specificity of the former was higher (81.25%) than the latter (68.75%). A separate analysis revealed that the Ki-67 index at a cut-off of 18% had a sensitivity of 80% and a specificity of 81.25% for predicting an unfavorable outcome.
摘要：
肾上腺皮质肿瘤在儿童时期很少见。它们的组织病理学分类为良性和恶性通常具有挑战性，影响进一步的管理。虽然AFIP/Wieneke评分系统广泛用于这些肿瘤的预后分类，它有局限性。在过去的几年中，很少有其他肿瘤评分系统得到发展。这些已经在成人中得到验证，但尚未在儿科患者中得到验证。我们评估了一组小儿肾上腺皮质肿瘤，以评估AFIP/Wieneke标准以及最近引入的赫尔辛基评分和网状蛋白算法在预测临床结果中的适用性。在存在以下任何一种情况下，肿瘤被认为是“临床上具有侵袭性”：转移，复发,进行性疾病,或因疾病而死亡。无任何此类事件的病例被认为“临床良好”。无事件生存时间是从临床表现日期到任何术后不良事件的持续时间。对于总体生存分析，终点是最后一次随访或因疾病死亡.采用ROC曲线分析,获得的截止赫尔辛基24分可以将病例分为两个预后相关组.生存分析显示两组患者的无事件生存率和总生存率存在显著差异,验证建议的截止值。三种组织病理学评分系统均无法以100％的准确性预测不利结果。全部显示出≥80%的灵敏度，与网状蛋白算法实现100%的灵敏度。AFIP/Wieneke标准的特异性和准确性最低(62.5%和73.08%,分别)。虽然赫尔辛基得分(在24分的截止分数)和网状蛋白算法具有相似的准确率(80.77%,80%,分别)，前者的特异性（81.25％）高于后者（68.75％）。另一项分析显示，Ki-67指数在临界值为18％时预测不利结果的敏感性为80％，特异性为81.25％。
