Abstract：
OBJECTIVE: Pediatric adrenocortical carcinoma (pACC) is rare, and prognostic stratification remains challenging. We aimed to confirm the prognostic value of the previously published pediatric scoring system (pS-GRAS) in an international multicenter cohort.
METHODS: Analysis of pS-GRAS items of pACC from 6 countries in collaboration of ENSAT-PACT, GPOH-MET, and IC-PACT.
METHODS: We received patient data of the pS-GRAS items including survival information from 9 centers. PS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2-3 = 1; 4 = 2 points), grade (Ki67 index: 0%-9% = 0; 10%-19% = 1; ≥20% = 2 points), resection status (R0 = 0; RX/R1/R2 = 1 point), age (<4 years = 0; ≥4 years = 1 point), and hormone production (androgen production = 0; glucocorticoid-/mixed-/no-hormone production = 1 point) generating 8 scores and 4 groups (1: 0-2, 2: 3-4, 3: 5, 4: 6-7). Primary endpoint was overall survival (OS).
RESULTS: We included 268 patients with median age of 4 years. The analysis of the pS-GRAS score showed a significantly favorable prognosis in patients with a lower scoring compared to higher scoring groups (5-year OS: Group 1 98%; group 2 87% [hazard ratio {HR} of death 3.6, 95% CI of HR 1.6-8.2]; group 3 43% [HR of death 2.8, 95% CI 1.9-4.4]; group 4: OS 18% [HR of death 2.1, 95% CI 1.7-2.7]). In the multivariable analysis, age (HR of death 3.5, 95% CI 1.8-7.0), resection status (HR of death 5.5, 95% CI 2.7-11.1), tumor stage (HR of death 1.9, 95% CI of HR 1.2-3.0), and Ki67 index (HR of death 1.7, 95% CI 1.2-2.4) remained strong independent outcome predictors. Especially infants < 4 years showed more often low-risk constellations with a better OS for all tumor stages.
CONCLUSIONS: In an international multicenter study, we confirmed that the pS-GRAS score is strongly associated with overall survival among patients with pACC. Age, resection status, stage, and Ki67 index are important parameters for risk stratification.
METHODS: Analysis of pS-GRAS items of pACC from 6 countries in collaboration of ENSAT-PACT, GPOH-MET, and IC-PACT.
METHODS: We received patient data of the pS-GRAS items including survival information from 9 centers. PS-GRAS score was calculated as a sum of tumor stage (1 = 0; 2-3 = 1; 4 = 2 points), grade (Ki67 index: 0%-9% = 0; 10%-19% = 1; ≥20% = 2 points), resection status (R0 = 0; RX/R1/R2 = 1 point), age (<4 years = 0; ≥4 years = 1 point), and hormone production (androgen production = 0; glucocorticoid-/mixed-/no-hormone production = 1 point) generating 8 scores and 4 groups (1: 0-2, 2: 3-4, 3: 5, 4: 6-7). Primary endpoint was overall survival (OS).
RESULTS: We included 268 patients with median age of 4 years. The analysis of the pS-GRAS score showed a significantly favorable prognosis in patients with a lower scoring compared to higher scoring groups (5-year OS: Group 1 98%; group 2 87% [hazard ratio {HR} of death 3.6, 95% CI of HR 1.6-8.2]; group 3 43% [HR of death 2.8, 95% CI 1.9-4.4]; group 4: OS 18% [HR of death 2.1, 95% CI 1.7-2.7]). In the multivariable analysis, age (HR of death 3.5, 95% CI 1.8-7.0), resection status (HR of death 5.5, 95% CI 2.7-11.1), tumor stage (HR of death 1.9, 95% CI of HR 1.2-3.0), and Ki67 index (HR of death 1.7, 95% CI 1.2-2.4) remained strong independent outcome predictors. Especially infants < 4 years showed more often low-risk constellations with a better OS for all tumor stages.
CONCLUSIONS: In an international multicenter study, we confirmed that the pS-GRAS score is strongly associated with overall survival among patients with pACC. Age, resection status, stage, and Ki67 index are important parameters for risk stratification.
摘要：
目的：小儿肾上腺皮质癌（pACC）很少见，预后分层仍然具有挑战性。我们旨在确认先前发表的儿科评分系统（pS-GRAS）在国际多中心队列中的预后价值。
方法：与ENSAT-PACT合作，对来自六个国家的pACC的pS-GRAS项目进行分析，GPOH-MET和IC-PACT。
方法：我们从9个中心接收了pS-GRAS项目的患者数据，包括生存信息。PS-GRAS评分计算为肿瘤分期的总和（1=0;2-3=1;4=2分），等级(Ki67指数:0-9%=0;10-19%=1;≥20%=2分),切除状态（R0=0;RX/R1/R2=1点），年龄(<4岁=0；≥4岁=1分)，和激素产生(雄激素产生=0;糖皮质激素-/混合/-无激素产生=1点)产生八个评分和四组(1:0-2,2:3-4,3:5,4:6-7)。主要终点是总生存期（OS）。
结果：我们纳入了268例患者，中位年龄为4岁。pS-GRAS评分分析显示，与较高评分组相比，评分较低的患者预后明显良好（5年OS：1组98％；2组87％（死亡HR3.6，HR95％CI1.6-8.2）；3组43％（死亡HR2.8，95％CI1.9-4.4）；4组：OS18％（死亡HR2.1，95％CI1.7-2.7））。在多变量分析年龄(死亡HR3.5，95%CI1.8-7.0)中，切除状态（死亡HR5.5，95%CI2.7-11.1），肿瘤分期(死亡HR1.9,95%-HR1.2-3.0的CI)和Ki67指数(死亡HR1.7,95%CI1.2-2.4)仍然是强有力的独立结局预测因子.特别是小于4岁的婴儿更经常显示低风险星座，所有肿瘤阶段的OS都更好。
结论：在一项国际多中心研究中，我们证实pS-GRAS评分与pACC患者的总生存期密切相关.年龄,切除状态,分期和Ki67指数是风险分层的重要参数。
方法：与ENSAT-PACT合作，对来自六个国家的pACC的pS-GRAS项目进行分析，GPOH-MET和IC-PACT。
方法：我们从9个中心接收了pS-GRAS项目的患者数据，包括生存信息。PS-GRAS评分计算为肿瘤分期的总和（1=0;2-3=1;4=2分），等级(Ki67指数:0-9%=0;10-19%=1;≥20%=2分),切除状态（R0=0;RX/R1/R2=1点），年龄(<4岁=0；≥4岁=1分)，和激素产生(雄激素产生=0;糖皮质激素-/混合/-无激素产生=1点)产生八个评分和四组(1:0-2,2:3-4,3:5,4:6-7)。主要终点是总生存期（OS）。
结果：我们纳入了268例患者，中位年龄为4岁。pS-GRAS评分分析显示，与较高评分组相比，评分较低的患者预后明显良好（5年OS：1组98％；2组87％（死亡HR3.6，HR95％CI1.6-8.2）；3组43％（死亡HR2.8，95％CI1.9-4.4）；4组：OS18％（死亡HR2.1，95％CI1.7-2.7））。在多变量分析年龄(死亡HR3.5，95%CI1.8-7.0)中，切除状态（死亡HR5.5，95%CI2.7-11.1），肿瘤分期(死亡HR1.9,95%-HR1.2-3.0的CI)和Ki67指数(死亡HR1.7,95%CI1.2-2.4)仍然是强有力的独立结局预测因子.特别是小于4岁的婴儿更经常显示低风险星座，所有肿瘤阶段的OS都更好。
结论：在一项国际多中心研究中，我们证实pS-GRAS评分与pACC患者的总生存期密切相关.年龄,切除状态,分期和Ki67指数是风险分层的重要参数。
