Adrenocortical tumors (ACT) in the adult and pediatric population are generally considered distinct entities due to differences in molecular events related to tumorigenesis, clinical presentation, and outcome. Furthermore, pathological criteria used for diagnosis and prognostication of ACT in adults are usually inadequate for predicting the biological behavior of ACT in children. Here, we analyzed 146 adult and 44 pediatric (< 15y/o) ACT with long-term clinical follow-up and furthered current evidence on the clinical and pathological differences between pediatric and adult tumors. Predilection for female over male gender was observed in both cohorts, but more so in adults (84% vs. 61%, p = 0.003). Cushing syndrome was more frequent in adults (p < 0.001), whereas virilization, either isolated (p < 0.001) or combined to Cushing (p = 0.047), was more common in children. The Ki67 labelling index (LI) of pediatric adenomas and carcinomas was much higher than their corresponding tumors in adults (p < 0.001). Despite these differences, pathological analyses including the evaluation of Ki67 greatly improved patient prognostication in both age cohorts. Indeed, increased Weiss scores and Ki67 indexes correlated with poor overall- and disease-free survival in adult patients with carcinoma. Among the proliferative indexes tested, Ki67 LI ≥ 10% showed the highest hazard ratio (HR) for recurrence and the Ki67 LI ≥ 3% showed the highest HR for survival. In pediatric tumors, the Wieneke score (p < 0.001) and the Ki67 LI (p < 0.001) showed high accuracy for predicting biological behavior, and increased scores/indexes correlated with worse overall and disease-free survival. In this age cohort, Ki67 LI < 10% was able to rule out malignant behavior, whereas Ki67 LI ≥ 15% may be used to predict the patients with higher risks of recurrence and/or poor outcome.

由于与肿瘤发生、临床表现和结果相关的分子事件的差异，成人和儿童人群中的肾上腺皮质肿瘤 (ACT) 通常被认为是不同的实体。此外，用于诊断和预测成人 ACT 的病理标准通常不足以预测儿童 ACT 的生物学行为。在这里，我们分析了 146 名成人和 44 名儿童（<15 岁/岁）ACT 的长期临床随访，并进一步证实了儿童和成人肿瘤之间临床和病理差异的当前证据。在两个队列中都观察到女性比男性的偏好，但在成年人中更是如此（84% 对 61%，p = 0.003）。库欣综合征在成人中更常见（p < 0.001），而男性化，无论是孤立的（p < 0.001）还是与库欣合并（p = 0.047），在儿童中更为常见。儿童腺瘤和癌的 Ki67 标记指数 (LI) 远高于成人相应肿瘤 (p < 0.001)。尽管存在这些差异，包括 Ki67 评估在内的病理分析极大地改善了两个年龄组的患者预后。事实上，增加的 Weiss 评分和 Ki67 指数与成年癌症患者的总体和无病生存率较差相关。在测试的增殖指标中，Ki67 LI ≥ 10% 显示出最高的复发风险比 (HR)，而 Ki67 LI ≥ 3% 显示出最高的生存率。在儿科肿瘤中，Wieneke 评分 (p < 0.001) 和 Ki67 LI (p < 0.001) 显示预测生物学行为的准确性很高，并且评分/指数的增加与较差的总体和无病生存率相关。