TY - JOUR
T1 - Diagnostics and treatment of diffuse intrinsic pontine glioma
T2 - where do we stand?
AU - El-Khouly, Fatma E
AU - Veldhuijzen van Zanten, Sophie E M
AU - Santa-Maria Lopez, Vicente
AU - Hendrikse, N Harry
AU - Kaspers, Gertjan J L
AU - Loizos, G
AU - Sumerauer, David
AU - Nysom, Karsten
AU - Pruunsild, Kaie
AU - Pentikainen, Virve
AU - Thorarinsdottir, Halldora K
AU - Rutkauskiene, Giedre
AU - Calvagna, Victor
AU - Drogosiewicz, Monika
AU - Dragomir, Monica
AU - Deak, Ladislav
AU - Kitanovski, Lidija
AU - von Bueren, Andre O
AU - Kebudi, Rejin
AU - Slavc, Irene
AU - Jacobs, Sandra
AU - Jadrijevic-Cvrlje, Filip
AU - Entz-Werle, Natacha
AU - Grill, Jacques
AU - Kattamis, Antonis
AU - Hauser, Peter
AU - Pears, Jane
AU - Biassoni, Veronica
AU - Massimino, Maura
AU - Lopez Aguilar, Enrique
AU - Torsvik, Ingrid K
AU - Joao Gil-da-Costa, Maria
AU - Kumirova, Ella
AU - Cruz-Martinez, Ofelia
AU - Holm, Stefan
AU - Bailey, Simon
AU - Hayden, Tim
AU - Thomale, Ulrich W
AU - Janssens, Geert O R
AU - Kramm, Christof M
AU - van Vuurden, Dannis G
PY - 2019/10
Y1 - 2019/10
N2 - 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.
AB - 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.
KW - Diffuse intrinsic pontine glioma (DIPG)
KW - Diffuse midline glioma H3-K27 mutant (DMG K3-27M)
KW - Chemotherapy
KW - Radiotherapy
UR - http://www.scopus.com/inward/record.url?scp=85072903436&partnerID=8YFLogxK
U2 - 10.1007/s11060-019-03287-9
DO - 10.1007/s11060-019-03287-9
M3 - Article
C2 - 31522324
SN - 0167-594X
VL - 145
SP - 177
EP - 184
JO - Journal of Neuro-Oncology
JF - Journal of Neuro-Oncology
IS - 1
ER -