Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium.
Department of Pediatric Hematology-Oncology, University Hospitals Leuven, Leuven, Belgium.
J Neurooncol. 2021 Jun;153(2):293-301. doi: 10.1007/s11060-021-03766-y. Epub 2021 May 3.
Diffuse intrinsic pontine glioma is a rare disease with a high mortality. Our primary aim was to determine the incidence of this disease in Belgium. Secondly, we wanted to compare the treatment approach of Belgian pediatric oncology centres, to investigate possibilities for improvement.
We retrospectively collected and analysed data on DIPG-patients diagnosed between 1994 and 2018 and recorded in the Belgian Cancer Registry. We included patients ≤ 18 years who were followed in one of the eight Belgian pediatric oncology centres.
We included 100 patients. Files were complete in 87 patients. We observed an increase in diagnoses with an incidence of 3.1 per 1,000,000 persons (aged 0-≤ 18) per year over the last 5 years compared to an overall incidence of 1.8. Biopsy was performed at diagnosis in 51.7% of patients. In one fifth this was study-related. Mutation analysis was known in eight patients, of which six showed the H3 K27M-mutation. 58.8% of patients received chemotherapy, without a significant survival benefit. 12.6% of patients were included in a clinical trial. Biopsy rate and the use of chemotherapy differed widely between centres. Mean OS and PFS were 10.49 and 4.87 months respectively. We observed an improved survival over time.
Over the past 25 years, we observed an increase of new DIPG-diagnoses. Outcome in our cohort is comparable with literature findings. We demonstrate an important heterogeneity in treatment approach between different centres and limited inclusion in clinical trials. Therefore, collaboration between centres and inclusion of patients in clinical trials is much needed.
弥漫性内生脑桥胶质瘤是一种死亡率很高的罕见疾病。我们的主要目的是确定该病在比利时的发病率。其次，我们希望比较比利时儿科肿瘤中心的治疗方法，以探讨改进的可能性。
我们回顾性地收集并分析了 1994 年至 2018 年间在比利时癌症登记处诊断为 DIPG 患者的数据，并记录在案。我们纳入了在 8 个比利时儿科肿瘤中心之一接受治疗且年龄≤18 岁的患者。
我们共纳入了 100 名患者。87 名患者的档案完整。与过去 5 年每年每 100 万人群中诊断出的 3.1 例（0-≤18 岁）相比，我们观察到诊断出的病例有所增加。51.7%的患者在诊断时进行了活检，其中五分之一是为了研究。在 8 名患者中已知存在突变分析，其中 6 名患者显示 H3 K27M 突变。58.8%的患者接受了化疗，但无显著生存获益。12.6%的患者参与了临床试验。各中心之间活检率和化疗的使用差异很大。中位 OS 和 PFS 分别为 10.49 个月和 4.87 个月。我们观察到随着时间的推移生存率有所提高。
在过去的 25 年中，我们观察到新诊断出的 DIPG 病例有所增加。我们队列的结果与文献发现相当。我们发现不同中心之间的治疗方法存在很大的异质性，且参与临床试验的人数有限。因此，各中心之间的合作和患者纳入临床试验是非常必要的。
