title: State of affairs in use of steroids in diffuse intrinsic pontine glioma: an international survey and a review of the literature.
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.
authors.affiliation: Division of Oncology/Haematology, Department of Paediatrics, VU University Medical Center (VUmc), De Boelelaan 1118, Room KTC4.027, 1081 HZ, Amsterdam, The Netherlands. s.veldhuijzen@vumc.nl.
authors.forename: Sophie E M
authors.initials: SE
authors.lastname: Veldhuijzen van Zanten
authors.affiliation: Department of Paediatric Oncology, Hospital Sant Joan de Déu (HSJD), Passeig Sant Joan de Déu, 2, Esplugues De Llobregat, 08950, Barcelona, Spain.
authors.forename: Ofelia
authors.initials: O
authors.lastname: Cruz
authors.affiliation: Division of Oncology/Haematology, Department of Paediatrics, VU University Medical Center (VUmc), De Boelelaan 1118, Room KTC4.027, 1081 HZ, Amsterdam, The Netherlands.|Princess Máxima Center of Pediatric Oncology, Lundlaan 6, 3584 EA, Utrecht, The Netherlands.
authors.forename: Gertjan J L
authors.initials: GJ
authors.lastname: Kaspers
authors.affiliation: Paediatric Oncology Unit, Great Ormond Street Hospital (GOSH), Great Ormond Street, London, WC1N 3JH, UK.
authors.forename: Darren R
authors.initials: DR
authors.lastname: Hargrave
authors.affiliation: Division of Oncology/Haematology, Department of Paediatrics, VU University Medical Center (VUmc), De Boelelaan 1118, Room KTC4.027, 1081 HZ, Amsterdam, The Netherlands.
authors.forename: Dannis G
authors.initials: DG
authors.lastname: van Vuurden
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D020295:Brain Stem Neoplasms / Q000188:drug therapy*; D005910:Glioma / Q000188:drug therapy*; D006282:Health Personnel; D006801:Humans; D038622:Internationality; D013256:Steroids / Q000009:adverse effects / Q000627:therapeutic use*; D011795:Surveys and Questionnaires
chemical_list: D000970:Antineoplastic Agents; D013256:Steroids
keywords: Diffuse Intrinsic Pontine Glioma (DIPG); Pediatric oncology; Quality of life (QoL); Side effects; Steroids