Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?

Autor: El-Khouly FE; Pediatric Oncology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands. f.el-khouly@amsterdamumc.nl.; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. f.el-khouly@amsterdamumc.nl.; Department of Pediatric Oncology/Hematology, Amsterdam UMC, location VUmc, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands. f.el-khouly@amsterdamumc.nl., Veldhuijzen van Zanten SEM; Pediatric Oncology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., Santa-Maria Lopez V; Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, St Joan de Déu Children´s Hospital, Barcelona, Spain., Hendrikse NH; Department of Clinical Pharmacology & Pharmacy, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.; Department of Radiology & Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands., Kaspers GJL; Pediatric Oncology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., Loizos G; Pediatric Oncology-Hematology Clinic, Archbishop Makarios III Hospital, Nicosia, Cyprus., Sumerauer D; Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, University Hospital Motol, Charles University, Prague, Czech Republic., Nysom K; Pediatrics and Adolescent Medicine, University Hospital Rigshospitalet, Copenhagen, Denmark., Pruunsild K; Department of Hematology and Oncology, Tallinn Children's Hospital, Tallinn, Estonia., Pentikainen V; Division of Hematology-Oncology and Stem Cell Transplantation, Children's Hospital, Helsinki University Hospital, Helsinki, Finland., Thorarinsdottir HK; Pediatric Hematology-Oncology, The Children's Hospital, Reykjavík, Iceland., Rutkauskiene G; Department of Pediatric Oncology and Hematology, Hospital of Lithuanian University of Health Sciences Kaunas Clinic, Kaunas, Lithuania., Calvagna V; Mater Dei Hospital, Valletta, Malta., Drogosiewicz M; Children's Memorial Health Institute, Warsaw, Poland., Dragomir M; Department of Pediatric Oncology, Oncology Institute Professor Doctor Alexandru Trestioreanu, Bucharest, Romania., Deak L; Department of Pediatric Oncology/Hematology, Children University Hospital, Kosice, Slovakia., Kitanovski L; Division of Hemato-Oncology, Department of Pediatrics, University Medical Center Ljubljana, Ljubljana, Slovenia., von Bueren AO; Pediatric Oncology and Hematology, Department of Pediatrics, University Hospital of Geneva, Geneva, Switzerland., Kebudi R; Pediatric Hematology-Oncology, Cerrahpasa Medical Faculty & Oncology Institute, Istanbul University, Istanbul, Turkey., Slavc I; Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria., Jacobs S; Universitair Ziekenhuis Leuven, Leuven, Belgium., Jadrijevic-Cvrlje F; Department of Oncology and Hematology, Children's Hospital Zagreb, Zagreb, Croatia., Entz-Werle N; CHRU Hautepierre Strasbourg, Service de Pédiatrie Onco-Hématologie, Strasbourg, France., Grill J; Département de Cancérologie de l'enfant et de l'adoloscent, CLCC Institut Gustave Roussy, Villejuif, France., Kattamis A; First Department of Pediatrics, 'Aghia Sofia' Children's Hospital, National and Kapodistrian University of Athens, Athens, Greece., Hauser P; Second Department of Pediatrics, Semmelweis University, Budapest, Hungary., Pears J; Department of Pediatric Oncology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland., Biassoni V; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy., Massimino M; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy., Lopez Aguilar E; Hospital de Pediatría, Centro Médico National Siglo XXI, Instituto Mexicano del Seguro Social, Jefatura de Servicio de Oncologia, Distrito Federal, Mexico., Torsvik IK; Division of Oncology/Hematology, Department of Pediatrics, Haukeland University Hospital, Mons, Norway., Joao Gil-da-Costa M; Pediatric Hematology and Oncology Division, University Hospital S. João Alameda Hernani Monteiro, Porto, Portugal., Kumirova E; Department of Neurooncology, Federal Research and Clinical Centre of Pediatric Hematology, Oncology and Immunology (FRC-PHOI), Moscow, Russia., Cruz-Martinez O; Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, St Joan de Déu Children´s Hospital, Barcelona, Spain., Holm S; Department of Pediatric Hematology and Oncology, Department of Woman and Child Health, Karolinska University Hospital, Stockholm, Sweden., Bailey S; Great North Children's Hospital, Victoria Wing, Royal Victoria Infirmary, Newcastle upon Tyne, UK., Hayden T; The DIPG Collaborative, Cincinnati, USA., Thomale UW; Pediatric Neurosurgery, Charité University Medical Center Berlin, Berlin, Germany., Janssens GOR; Department of Radiation Oncology, University Medical Center Utrecht, Utrecht, The Netherlands., Kramm CM; Division of Pediatric Hematology and Oncology, University Medical Center Geottingen, Göttingen, Germany., van Vuurden DG; Pediatric Oncology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Jazyk: angličtina
Zdroj: Journal of neuro-oncology [J Neurooncol] 2019 Oct; Vol. 145 (1), pp. 177-184. Date of Electronic Publication: 2019 Sep 14.
DOI: 10.1007/s11060-019-03287-9
Abstrakt: 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.
Databáze: MEDLINE
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