Napieralska Aleksandra, Mizia-Malarz Agnieszka, Stolpa Weronika, Pawłowska Ewa, Krawczyk Małgorzata A, Konat-Bąska Katarzyna, Kaczorowska Aneta, Brąszewski Arkadiusz, Harat Maciej
Department of Radiotherapy, Maria Sklodowska-Curie National Research Institute of Oncology Gliwice Branch, 44-101 Gliwice, Poland.
Department of Pediatrics, Medical University of Silesia, 40-752 Katowice, Poland.
Diagnostics (Basel). 2021 Dec 14;11(12):2360. doi: 10.3390/diagnostics11122360.
We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan-Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/-CTH afterwards; = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS ( = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.
我们对74例室管膜瘤患儿进行了多机构分析,以评估前瞻性试验的临床结果在常规实践中能够重现的程度。采用对数秩检验和Cox比例风险模型对与预后相关的因素进行评估。采用Kaplan-Meier法估计生存率。大多数患者患有脑肿瘤(89%)。所有患者均以手术作为主要治疗手段,分别有78%和57%的患者接受了辅助放疗(RTH)和化疗(CTH)。中位随访时间为80个月,18例患者死亡。5年和10年总生存率(OS)分别为83%和73%。32例患者出现病情进展,其中28例为局部复发。转移的存在是OS的不良预后因素。5年和10年无进展生存率(PFS)分别为55%和40%。对于未播散性脑肿瘤患者,手术后继以RTH(之后可±CTH)时观察到最佳预后(=0.0001)。在初始治疗中接受RTH的3岁以下儿童具有更好的PFS(=0.010)。室管膜瘤患儿的最佳预后见于接受根治性手术后继以RTH的患者,且对于较年轻患者不应省略放疗。CTH的作用仍存在争议。