Introduction: Adrenocortical carcinoma (ACC) is diagnosed in paediatric patients at 5 months after symptom onset on average, and 38% die during the first 2.5 years of follow-up. This study aimed to compare the accuracy of Weiss, Van Slooten, and Wieneke histopathological ACC classifications for predicting follow-up prognosis in a paediatric population. Methods: Data were retrieved from medical records of 57 patients aged <18 years who underwent surgical treatment for ACC with surgical follow-up over 6 months or death due to ACC. They were classified into either good (without recurrence/death due to ACC) or poor (with recurrence/death due to ACC) prognosis group. Two expert pathologists classified the ACC surgical specimens according to the Weiss, Van Slooten, and Wieneke criteria. Results: The median follow-up duration was 126 [18-225] months in 38 males (66.7%) and 19 females (33.3%) (median age: 3 [1-6.5] years). The good prognosis group was younger than the poor prognosis group (median age: 3 [1.5-6.2] years vs. 5 [2-10] years). Seventeen (29.8%) patients in the poor prognosis group died due to ACC within the first 50 months of surgical follow-up; the earliest death occurred in the fourth follow-up month, and the majority of deaths occurred within 24 months of follow-up. The accuracies of Weiss, Van Slooten, and Wieneke classification systems were 40%, 47%, and 77%, respectively. Discussion/Conclusion: The Wieneke classification showed the best accuracy but was not sufficiently precise to establish reliable prognosis for ACC in the paediatric population. The Wieneke classification had approximately 95% sensitivity and negative predictive value.


中文翻译：

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小儿肾上腺皮质癌的组织病理学标准

简介：儿科患者平均在症状出现后 5 个月诊断出肾上腺皮质癌 (ACC)，38% 的患者在随访的前 2.5 年内死亡。本研究旨在比较 Weiss、Van Slooten 和 Wieneke 组织病理学 ACC 分类预测儿科人群随访预后的准确性。方法：从 57 名年龄 <18 岁接受 ACC 手术治疗且手术随访超过 6 个月或因 ACC 死亡的患者的医疗记录中检索数据。他们被分为良好（没有因 ACC 复发/死亡）或不良（因 ACC 复发/死亡）预后组。两位病理学家根据 Weiss、Van Slooten 和 Wieneke 标准对 ACC 手术标本进行分类。结果：38 名男性 (66.7%) 和 19 名女性 (33.3%) 的中位随访时间为 126 [18-225] 个月（中位年龄：3 [1-6.5] 岁）。预后良好组比预后不良组年轻（中位年龄：3 [1.5-6.2] 岁对 5 [2-10] 岁）。预后不良组中有 17 名 (29.8%) 患者在手术随访的前 50 个月内死于 ACC；最早的死亡发生在随访的第四个月，大多数死亡发生在随访的 24 个月内。Weiss、Van Slooten 和 Wieneke 分类系统的准确度分别为 40%、47% 和 77%。讨论/结论：Wieneke 分类显示出最佳的准确性，但不够精确，无法在儿科人群中建立可靠的 ACC 预后。