Bergadá I, Venara M, Maglio S, Ciaccio M, Diez B, Bergadá C, Chemes H
Division Endocrinologia, Hospital de Ninos "R. Gutiérrez,¿ Buenos Aires, Argentina.
Cancer. 1996 Feb 15;77(4):771-7.
Controversy exists as to which variable is a reliable predictor of clinical outcome of adrenal cortical tumors in children.
Twenty patients with adrenal cortical tumors were studied. Tumor weight, histologic features, and percentage of proliferating cell nuclear antigen (PCNA/cyclin) in tumor cells were analyzed to determine the best predictor of clinical outcome.
Eleven patients had Cushing's syndrome with virilization and 9 had virilization without Cushing's syndrome. The mean age at diagnosis was 7.1 +/- 5.2 years (range, 0.4-15.6 years). Sixteen patients, with good outcomes have been followed for 10.7 +/- 7.8 years (range, 3-23 years). All but two patients had a tumor weight of less than 100 g (185 g and 800 g, respectively) (mean 47.7 g +/- 46.4 g). Two patients with large tumors (weighing 1000 g and 780 g, respectively) had poor outcomes; 1 died 3 months after surgery with metastasis and the other presented with lung metastasis 18 months after surgery. Histologic features did not correlate with clinical outcome. Overall, PCNA stained cells were 6.96 +/- 8.2% (range, 0-32.5%). PCNA values were significantly lower in tumors of patients with good outcomes (P < 0.002). Within all tumors, we found a weak correlation between tumor weight and PCNA (r = 0.51; P < 0.02), but a better correlation was found between tumor weight and PCNA in patients with Cushing's syndrome (r = 0.70; P < 0.01). Patients with Cushing's syndrome had higher PCNA values than those with virilization syndrome (10.3 +/- 9.6% vs. 2.8 +/- 3.3%; P < 0.03).
Our data show that small tumors (less than 100 g) are associated with good outcome; the two patients with the poorest prognosis had Cushing's syndrome and large tumors (more than 100 g). Histologic features are not adequate predictors of outcome and PCNA may be useful in tumors of patients with Cushing's syndrome, but this parameter should not be used alone. Two patients had virilization syndrome, large tumors (185 g and 800 g, respectively), and good outcomes, which contradicts with the concept that these tumors are usually associated with poor prognosis.
关于哪个变量可作为儿童肾上腺皮质肿瘤临床结局的可靠预测指标，目前仍存在争议。
对20例肾上腺皮质肿瘤患者进行研究。分析肿瘤重量、组织学特征以及肿瘤细胞中增殖细胞核抗原（PCNA/细胞周期蛋白）的百分比，以确定临床结局的最佳预测指标。
11例患者患有库欣综合征伴男性化，9例患者仅有男性化而无库欣综合征。诊断时的平均年龄为7.1±5.2岁（范围0.4 - 15.6岁）。16例预后良好的患者随访了10.7±7.8年（范围3 - 23年）。除2例患者外，所有患者的肿瘤重量均小于100 g（分别为185 g和800 g）（平均47.7 g±46.4 g）。2例肿瘤较大的患者（分别重1000 g和780 g）预后较差；1例术后3个月因转移死亡，另1例术后18个月出现肺转移。组织学特征与临床结局无关。总体而言，PCNA染色细胞为6.96±8.2%（范围0 - 32.5%）。预后良好患者的肿瘤中PCNA值显著更低（P < 0.002）。在所有肿瘤中，我们发现肿瘤重量与PCNA之间存在弱相关性（r = 0.51；P < 0.02），但在库欣综合征患者的肿瘤中，肿瘤重量与PCNA之间的相关性更好（r = 0.70；P < 0.01）。库欣综合征患者的PCNA值高于男性化综合征患者（10.3±9.6%对2.8±3.3%；P < 0.03）。
我们的数据表明，小肿瘤（小于100 g）与良好结局相关；预后最差的2例患者患有库欣综合征且肿瘤较大（大于100 g）。组织学特征不足以预测结局，PCNA在库欣综合征患者的肿瘤中可能有用，但该参数不应单独使用。2例患有男性化综合征、肿瘤较大（分别为185 g和800 g）且预后良好的患者，这与这些肿瘤通常预后较差的概念相矛盾。
