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Abstract
Introduction: Children with diffuse intrinsic pontine glioma (DIPG) face a dismal prognosis, with a median overall survival of 9 months. Our aims are to determine the incidence of DIPG in the Netherlands and to identify points for improvement in clinical research, a prerequisite for increasing the chance to find a cure. Methods: We performed a population-based retrospective cohort study by evaluating all children diagnosed with DIPG in the Netherlands between 1990 and 2010. Results: The incidence of DIPG in the Netherlands corresponds with international literature. Between 1990 and 2010, a large heterogeneity of treatment schedules was applied and only a minority of patients was included in clinical trials. Discussion: Given the rarity of DIPG, we emphasize the need for (inter-)national trials to facilitate the identification of potentially effective therapeutics in the future. This can be supported by the recent development of a European DIPG registry enabling international study collaborations.
Financial & competing interests disclosure
Diffuse intrinsic pontine glioma research in our institute was possible due to financial support from the Semmy Foundation (Stichting Semmy), and also by the Egbers Foundation (Egbers Stichting), as well as by the Children Cancer-free foundation (KiKa) and VONK (VUmc research on Childhood Cancer). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.
No writing assistance was utilized in the production of this manuscript.
Patients with diffuse intrinsic pontine glioma (DIPG) face a dismal prognosis, with a median overall survival of 9 months.
In recent decades, the survival has not improved despite several treatment strategies that have been explored.
The incidence of DIPG in the Netherlands is 9 (5–13) patients per year (0.54 per 1,000,000; 2.32 per 1,000,000 aged 0–20 years).
Between 1990 and 2010, for both radio- and chemotherapy, more than 10 different schedules or agents were used.
In this period, only 18% of patients were formally included in clinical trials.
Poor accrual of DIPG patients in clinical trials results in a lack of comprehensive data on demographics, history, physical examination, diagnosis (both imaging and biology), outcome, but most importantly effectiveness per treatment schedule, drug or dosage.
Given the rarity of DIPG, we emphasize the need for (inter-)national trials to facilitate the identification of potentially effective therapeutics in the future.
Recently, a European DIPG registry Citation[25] has been developed by the DIPG network of the International Society of Pediatric Oncology Europe. This registry will enable the evaluation and follow-up of clinical and centrally reviewed radiology data of all European patients with DIPG, both in- and outside clinical trials, which will give a realistic picture of the actual spectrum of DIPG patients. Subsequently, a comprehensive European DIPG registry will pave the way for further international collaborations and, hopefully, European clinical DIPG trials.