Ronsley Rebecca, Crowell Cameron, Irvine Mike, Kang Mehima, Goldman Ran D, Erker Craig, Cheng Sylvia
Division of Hematology, Oncology and Bone Marrow Transplant, Department of Pediatrics, British Columbia Children's Hospital.
Division of Hematology/Oncology, Department of Pediatrics, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada.
J Pediatr Hematol Oncol. 2023 Mar 1;45(2):e188-e193. doi: 10.1097/MPH.0000000000002424. Epub 2022 Feb 4.
The aim was to determine the impact of time to diagnosis (TTD) on morbidity and mortality and to identify factors associated with overall survival (OS) in pediatric patients with malignant central nervous system (CNS) tumors.
This is a retrospective review of all malignant CNS tumors presenting to 2 tertiary care pediatric hospitals from 2000 to 2019. Cox proportional hazard model analysis outcomes included TTD and OS as well as morbidity; stratified by tumor category, age, relapse, and presence of metastatic disease.
There were 197 children with malignant CNS tumors (mean age 8.7 y, 61% male). Tumors included medulloblastoma (N=58, 29.4%), ependymoma (N=27, 13.7%), high-grade glioma (N=42, 21.3%), germ cell tumors (N=47, 23.9%), and other embryonal tumors (N=23, 11.7%). Median TTD from symptom onset was 62 (interquartile range: 26.5 to 237.5 d) and 28% had metastatic disease. Three-year progression free survival was 55% and 3-year OS was 73.1%. Increased OS was associated with increased TTD (parameter estimate 0.12; confidence interval [CI]: 0.019-7.06; P =0.019), high-grade glioma (hazard ratio [HR]: 2.46; CI [1.03-5.86]; P =0.042), other embryonal tumor (HR: 2.84; CI [1.06-7.56]; P =0.037), relapse (HR: 10.14; CI: 4.52-22.70; P <0.001) and metastatic disease (HR: 3.25; CI: 1.51-6.96; P =0.002). Vision change (HR: 0.58; CI: 0.313-1.06; P =0.078), hearing loss (HR: 0.71; CI: 0.35-1.42; P =0.355), and cognitive impairment (HR: 0.73; CI: 0.45-1.19; P =0.205) were not associated with TTD in this model.
Increased median TTD is associated with higher OS in pediatric patients treated for malignant CNS tumors. Tumor biology and treatment modality are more important factors than TTD for predicting morbidity and long-term outcomes in pediatric patients with CNS tumors.
本研究旨在确定诊断时间（TTD）对小儿恶性中枢神经系统（CNS）肿瘤患者发病率和死亡率的影响，并确定与总生存期（OS）相关的因素。
这是一项对2000年至2019年在两家三级儿科专科医院就诊的所有恶性CNS肿瘤患者的回顾性研究。Cox比例风险模型分析的结果包括TTD、OS以及发病率；按肿瘤类别、年龄、复发情况和是否存在转移性疾病进行分层。
共有197例小儿恶性CNS肿瘤患者（平均年龄8.7岁，61%为男性）。肿瘤包括髓母细胞瘤（N = 58，29.4%）、室管膜瘤（N = 27，13.7%）、高级别胶质瘤（N = 42，21.3%）、生殖细胞瘤（N = 47，23.9%）和其他胚胎性肿瘤（N = 23，11.7%）。从症状出现到诊断的中位TTD为62天（四分位间距：26.5至237.5天），28%的患者有转移性疾病。三年无进展生存率为55%，三年总生存率为73.1%。总生存期的增加与诊断时间的增加相关（参数估计值0.12；置信区间[CI]：0.019 - 7.06；P = 0.019）、高级别胶质瘤（风险比[HR]：2.46；CI[1.03 - 5.86]；P = 0.042）、其他胚胎性肿瘤（HR：2.84；CI[1.06 - 7.56]；P = 0.037）、复发（HR：10.14；CI：4.52 - 22.70；P < 0.001）和转移性疾病（HR：3.25；CI：1.51 - 6.96；P = 0.002）。在该模型中，视力改变（HR：0.58；CI：0.313 - 1.06；P = 0.078）、听力丧失（HR：0.71；CI：0.35 - 1.42；P = 0.355）和认知障碍（HR：0.73；CI：0.45 - 1.19；P = 0.205）与TTD无关。
在接受治疗的小儿恶性CNS肿瘤患者中，中位TTD的增加与较高的总生存期相关。对于预测小儿CNS肿瘤患者的发病率和长期预后，肿瘤生物学和治疗方式比TTD更重要。
