INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines. METHODS: Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively. RESULTS: Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials. CONCLUSION: This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

1st Department of Pediatrics 'Aghia Sofia' Children's Hospital National and Kapodistrian University of Athens Athens Greece

2nd Department of Pediatrics Semmelweis University Budapest Hungary

Children's Memorial Health Institute Warsaw Poland

CHRU Hautepierre Strasbourg Service de Pédiatrie Onco Hématologie Strasbourg France

Département de Cancérologie de l'enfant et de l'adoloscent CLCC Institut Gustave Roussy Villejuif France

Department of Clinical Pharmacology and Pharmacy Amsterdam UMC Vrije Universiteit Amsterdam Amsterdam The Netherlands

Department of Hematology and Oncology Tallinn Children's Hospital Tallinn Estonia

Department of Neurooncology Federal Research and Clinical Centre of Pediatric Hematology Oncology and Immunology Moscow Russia

Department of Oncology and Hematology Children's Hospital Zagreb Zagreb Croatia

Department of Pediatric Hematology and Oncology 2nd Faculty of Medicine University Hospital Motol Charles University Prague Czech Republic

Department of Pediatric Hematology and Oncology Department of Woman and Child Health Karolinska University Hospital Stockholm Sweden

Department of Pediatric Hematology Oncology and Stem Cell Transplantation St Joan de Déu Children´s Hospital Barcelona Spain

Department of Pediatric Oncology and Hematology Hospital of Lithuanian University of Health Sciences Kaunas Clinic Kaunas Lithuania

Department of Pediatric Oncology Hematology Amsterdam UMC location VUmc De Boelelaan 1117 1081 HV Amsterdam The Netherlands

Department of Pediatric Oncology Hematology Children University Hospital Kosice Slovakia

Department of Pediatric Oncology Oncology Institute Professor Doctor Alexandru Trestioreanu Bucharest Romania

Department of Pediatric Oncology Our Lady's Children's Hospital Crumlin Dublin Ireland

Department of Pediatrics and Adolescent Medicine Medical University of Vienna Vienna Austria

Department of Radiation Oncology University Medical Center Utrecht Utrecht The Netherlands

Department of Radiology and Nuclear Medicine Amsterdam UMC Vrije Universiteit Amsterdam Amsterdam The Netherlands

Division of Hemato Oncology Department of Pediatrics University Medical Center Ljubljana Ljubljana Slovenia

Division of Hematology Oncology and Stem Cell Transplantation Children's Hospital Helsinki University Hospital Helsinki Finland

Division of Oncology Hematology Department of Pediatrics Haukeland University Hospital Mons Norway

Division of Pediatric Hematology and Oncology University Medical Center Geottingen Göttingen Germany

Great North Children's Hospital Victoria Wing Royal Victoria Infirmary Newcastle upon Tyne UK

Hospital de Pediatría Centro Médico National Siglo XXI Instituto Mexicano del Seguro Social Jefatura de Servicio de Oncologia Distrito Federal Mexico

Mater Dei Hospital Valletta Malta

Pediatric Hematology and Oncology Division University Hospital S João Alameda Hernani Monteiro Porto Portugal

Pediatric Hematology Oncology Cerrahpasa Medical Faculty and Oncology Institute Istanbul University Istanbul Turkey

Pediatric Hematology Oncology The Children's Hospital Reykjavík Iceland

Pediatric Neurosurgery Charité University Medical Center Berlin Berlin Germany

Pediatric Oncology and Hematology Department of Pediatrics University Hospital of Geneva Geneva Switzerland

Pediatric Oncology Emma Children's Hospital Amsterdam UMC Vrije Universiteit Amsterdam Amsterdam The Netherlands

Pediatric Oncology Hematology Clinic Archbishop Makarios 3 Hospital Nicosia Cyprus

Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale dei Tumori Milan Italy

Pediatrics and Adolescent Medicine University Hospital Rigshospitalet Copenhagen Denmark

Princess Máxima Center for Pediatric Oncology Utrecht The Netherlands

The DIPG Collaborative Cincinnati USA

Universitair Ziekenhuis Leuven Leuven Belgium

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Personalized Treatment of H3K27M-Mutant Pediatric Diffuse Gliomas Provides Improved Therapeutic Opportunities