Sakoda Akiko, Mushtaq Imran, Levitt Gill, Sebire Neil J
Department of Paediatric Urology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK.
Department of Oncology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK.
J Pediatr Surg. 2014 Mar;49(3):410-5. doi: 10.1016/j.jpedsurg.2013.09.008.
BACKGROUND/PURPOSE: Adrenocortical tumors (ACTs) are rare in children and the extent to which histopathological features can predict clinical behavior remains uncertain. The aim of this study was to investigate the relationship between histopathological features (Weiss score), surgical approach, tumor size, underlying genetic predisposition syndrome, and outcome.
Twenty-nine ACTs treated at our institution between 1987 and 2011 were identified from a histopathology database. The histological features were categorized using the Weiss scoring system. For tumor staging, the UKCCSG staging system was utilized.
At a median follow-up of 25 months, 19 patients (65.5%) survived without evidence of disease and 10 patients (35.5%) had died. There was a strong association between high Weiss score and both large tumor size (P<0.01) and adverse outcome (P<0.01). Outcome for stage I and IIA disease was significantly better compared to higher stage disease and/or tumor rupture (P<0.01).
There is an association between high Weiss score, large tumor size, underlying genetic predisposition syndrome and an adverse outcome for pediatric ACTs. Regardless of histopathological findings, complete surgical resection, without tumor spillage, is optimal for survival. Genetic evaluation is recommended in patients with ACTs, particularly those with a high Weiss score.
背景/目的：肾上腺皮质肿瘤（ACTs）在儿童中较为罕见，组织病理学特征对临床行为的预测程度仍不明确。本研究旨在探讨组织病理学特征（Weiss评分）、手术方式、肿瘤大小、潜在的遗传易感性综合征与预后之间的关系。
从组织病理学数据库中识别出1987年至2011年在本机构接受治疗的29例ACTs。使用Weiss评分系统对组织学特征进行分类。对于肿瘤分期，采用UKCCSG分期系统。
中位随访25个月时，19例患者（65.5%）存活且无疾病证据，10例患者（35.5%）死亡。高Weiss评分与大肿瘤大小（P<0.01）和不良预后（P<0.01）之间均存在密切关联。I期和IIA期疾病的预后明显优于更高分期疾病和/或肿瘤破裂（P<0.01）。
高Weiss评分、大肿瘤大小、潜在的遗传易感性综合征与儿童ACTs的不良预后之间存在关联。无论组织病理学结果如何，完整的手术切除且无肿瘤溢出对生存最为有利。建议对ACTs患者进行基因评估，尤其是那些Weiss评分高的患者。
