Abstract
Children diagnosed with diffuse intrinsic pontine glioma (DIPG) face a dismal prognosis, with severe neurologic deterioration and inevitable death at a median of 9 months from diagnosis. Steroids are widely prescribed as supportive or palliative treatment although they are known to cause severe side effects that may reduce the quality of life. This study aims to review the current knowledge on, and use of, steroids in DIPG patients. A global questionnaire-study among health care professionals was performed to ascertain information on the current (multi-)institutional and (multi-)national use of steroids, the availability of clinical guidelines, and the need for improvements in prescribing steroids to DIPG patients. In addition, an extensive literature search was performed to review studies investigating steroids in pediatric brain tumor patients. From 150 responding health care professionals, only 7 % had clinical guidelines. The use of steroids was heterogeneous and over 85 % of respondents reported serious side effects. Fourteen articles, with low level of evidence, described the use of steroids in pediatric brain tumor patients. Clinical trials investigating optimal dose or regimen were lacking. This study is a first inventory of the availability of evidence-based information and clinical guidelines, and the current attitude towards the use of steroids in DIPG patients. To date, the risk–benefit ratio of steroids in this disease is yet to be determined. We emphasize the need for clinical trials resulting in guidelines on steroids, and possibly alternative drugs, to optimize the quality of care and quality of life of DIPG patients.Electronic supplementary materialThe online version of this article (doi:10.1007/s11060-016-2141-x) contains supplementary material, which is available to authorized users.
Highlights
Diffuse intrinsic pontine glioma (DIPG) is a childhood brain tumor that grows diffusely in between the critical structures of the brainstem
It was not possible to determine the response rate from the electronic mailing lists of the Society of Paediatric Oncology Europe (SIOPE), International Society of Paediatric Neuro-oncology (ISPNO) and the International Brain Tumor Alliance (IBTA), as (1) it was unclear how many professionals were included, (2) these lists include professionals not directly involved in the treatment of diffuse intrinsic pontine glioma (DIPG) patients, and (3) professionals were asked to forward this invitation to the survey to colleagues within their institution and/or national groups
Our extensive worldwide survey with 150 respondents from all over the world provides a good overview of the current use of steroids in DIPG patients
Summary
Diffuse intrinsic pontine glioma (DIPG) is a childhood brain tumor that grows diffusely in between the critical structures of the brainstem. Patients have a two-year survival rate of less than 10 % [1]. Given the poor prognosis and lack of effective treatment options, maintenance of a good quality of life for as long as possible should be a major goal in the management approach of DIPG patients. Steroids are widely prescribed as supportive or palliative treatment. They are, well known to cause numerous side effects, which in turn may comprise the patient’s quality of life. Little research into the risk–benefit ratio and use of steroids in DIPG patients has been performed
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