Sabaretnam M, Mishra A, Agarwal G, Agarwal A, Verma A K, Mishra S K
Department of Endocrine Surgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
Indian J Cancer. 2016 Apr-Jun;53(2):317-321. doi: 10.4103/0019-509X.197737.
Adrenocortical carcinoma (ACC) occurring in children and adults show distinct characteristics. However, due to rarity of the disease no large series addressing this issue has been published.
The aim of this study was to study clinico-pathologic profile and outcome of ACC in children and adults.
Tertiary referral center. Retrospective study (January 1990-June 2011).
Forty-five patients with ACC were included; 16children (aged < 18 years) and 29 adults. Clinical details, hormonal profile, operation records, pathology reports and follow-up findings were noted and compared. Survival analysis was performed using Kaplan-Meier method. Log rank test and Cox regressionan alysis were performed.
Mean age was 8 ± 5.7 (M: F = 1:2.1) in children and 44.4 ± 15 years (M: F = 1:1.1) in adult groups. Prevalence of functioning tumors was significantly high in children (87.5 vs. 31% P = 0.001), while prevalence of incidentalomas was high in adults (6.3 vs. 51.7% P = 0.05). Tumor stage distribution at presentation, mean diameter (10.9 vs. 13.7 cm), and weight (392.9 vs. 892.9 g) didn't differ significantly in two groups. Adults had better albeit non-significant 5 year overall survival (OS) than children (0 vs. 13%). On univariate analysis stage of disease (P = 0.008), surgical intervention (P = 0.004), Weiss score (P = 0.04) and hormonal secretion (P = 0.04) were significantly associated with OS in adults but not in children. No factor was found significant on multivariate analysis.
Except for high prevalence of functioning tumors in children, clinico-pathologic attributes and outcome of ACC in the two groups didn't differ significantly.
儿童和成人肾上腺皮质癌（ACC）表现出不同的特征。然而，由于该疾病罕见，尚未发表针对此问题的大型系列研究。
本研究旨在探讨儿童和成人ACC的临床病理特征及预后。
三级转诊中心。回顾性研究（1990年1月至2011年6月）。
纳入45例ACC患者，其中16例儿童（年龄<18岁），29例成人。记录并比较临床细节、激素水平、手术记录、病理报告及随访结果。采用Kaplan-Meier法进行生存分析，并行对数秩检验和Cox回归分析。
儿童组平均年龄为8±5.7岁（男：女=1：2.1），成人组为44.4±15岁（男：女=1：1.1）。儿童组功能性肿瘤的患病率显著高于成人（87.5%对31%，P=0.001），而成人组偶发瘤的患病率较高（6.3%对51.7%，P=0.05）。两组患者初诊时肿瘤分期分布、平均直径（10.9对13.7 cm）和重量（392.9对892.9 g）差异无统计学意义。成人5年总生存率（OS）虽略高于儿童，但差异无统计学意义（0对13%）。单因素分析显示，疾病分期（P=0.008）、手术干预（P=0.004）、Weiss评分（P=0.04）和激素分泌（P=0.04）与成人OS显著相关，但与儿童OS无关。多因素分析未发现显著因素。
除儿童功能性肿瘤患病率较高外，两组ACC的临床病理特征及预后差异无统计学意义。
