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

Fatma E. el-Khouly, Sophie E. M. Veldhuijzen van Zanten, Vicente Santa-Maria Lopez, N. Harry Hendrikse, Gertjan J. L. Kaspers, G. Loizos, David Sumerauer, Karsten Nysom, Kaie Pruunsild, Virve Pentikainen, Halldora K. Thorarinsdottir, Giedre Rutkauskiene, Victor Calvagna, Monika Drogosiewicz, Monica Dragomir, Ladislav Deak, Lidija Kitanovski, Andre O. von Bueren, Rejin Kebudi, Irene Slavc & 21 others Sandra Jacobs, Filip Jadrijevic-Cvrlje, Natacha Entz-Werle, Jacques Grill, Antonis Kattamis, Peter Hauser, Jane Pears, Veronica Biassoni, Maura Massimino, Enrique Lopez Aguilar, Ingrid K. Torsvik, Maria Joao Gil-da-Costa, Ella Kumirova, Ofelia Cruz-Martinez, Stefan Holm, Simon Bailey, Tim Hayden, Ulrich W. Thomale, Geert O. R. Janssens, Christof M. Kramm, Dannis G. van Vuurden

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

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.
Original languageEnglish
Pages (from-to)177-184
Number of pages8
JournalJournal of Neuro-Oncology
Volume145
Issue number1
Early online date14 Sep 2019
DOIs
Publication statusPublished - 2019

Cite this

el-Khouly, Fatma E. ; Veldhuijzen van Zanten, Sophie E. M. ; Santa-Maria Lopez, Vicente ; Hendrikse, N. Harry ; Kaspers, Gertjan J. L. ; Loizos, G. ; Sumerauer, David ; Nysom, Karsten ; Pruunsild, Kaie ; Pentikainen, Virve ; Thorarinsdottir, Halldora K. ; Rutkauskiene, Giedre ; Calvagna, Victor ; Drogosiewicz, Monika ; Dragomir, Monica ; Deak, Ladislav ; Kitanovski, Lidija ; von Bueren, Andre O. ; Kebudi, Rejin ; Slavc, Irene ; Jacobs, Sandra ; Jadrijevic-Cvrlje, Filip ; Entz-Werle, Natacha ; Grill, Jacques ; Kattamis, Antonis ; Hauser, Peter ; Pears, Jane ; Biassoni, Veronica ; Massimino, Maura ; Lopez Aguilar, Enrique ; Torsvik, Ingrid K. ; Joao Gil-da-Costa, Maria ; Kumirova, Ella ; Cruz-Martinez, Ofelia ; Holm, Stefan ; Bailey, Simon ; Hayden, Tim ; Thomale, Ulrich W. ; Janssens, Geert O. R. ; Kramm, Christof M. ; van Vuurden, Dannis G. / Diagnostics and treatment of diffuse intrinsic pontine glioma : where do we stand?. In: Journal of Neuro-Oncology. 2019 ; Vol. 145, No. 1. pp. 177-184.
@article{e13e917b880e4eb582f2bae1069f6406,
title = "Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?",
abstract = "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.",
author = "el-Khouly, {Fatma E.} and {Veldhuijzen van Zanten}, {Sophie E. M.} and {Santa-Maria Lopez}, Vicente and Hendrikse, {N. Harry} and Kaspers, {Gertjan J. L.} and G. Loizos and David Sumerauer and Karsten Nysom and Kaie Pruunsild and Virve Pentikainen and Thorarinsdottir, {Halldora K.} and Giedre Rutkauskiene and Victor Calvagna and Monika Drogosiewicz and Monica Dragomir and Ladislav Deak and Lidija Kitanovski and {von Bueren}, {Andre O.} and Rejin Kebudi and Irene Slavc and Sandra Jacobs and Filip Jadrijevic-Cvrlje and Natacha Entz-Werle and Jacques Grill and Antonis Kattamis and Peter Hauser and Jane Pears and Veronica Biassoni and Maura Massimino and {Lopez Aguilar}, Enrique and Torsvik, {Ingrid K.} and {Joao Gil-da-Costa}, Maria and Ella Kumirova and Ofelia Cruz-Martinez and Stefan Holm and Simon Bailey and Tim Hayden and Thomale, {Ulrich W.} and Janssens, {Geert O. R.} and Kramm, {Christof M.} and {van Vuurden}, {Dannis G.}",
year = "2019",
doi = "10.1007/s11060-019-03287-9",
language = "English",
volume = "145",
pages = "177--184",
journal = "Journal of Neuro-Oncology",
issn = "0167-594X",
publisher = "Kluwer Academic Publishers",
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el-Khouly, FE, Veldhuijzen van Zanten, SEM, Santa-Maria Lopez, V, Hendrikse, NH, Kaspers, GJL, Loizos, G, Sumerauer, D, Nysom, K, Pruunsild, K, Pentikainen, V, Thorarinsdottir, HK, Rutkauskiene, G, Calvagna, V, Drogosiewicz, M, Dragomir, M, Deak, L, Kitanovski, L, von Bueren, AO, Kebudi, R, Slavc, I, Jacobs, S, Jadrijevic-Cvrlje, F, Entz-Werle, N, Grill, J, Kattamis, A, Hauser, P, Pears, J, Biassoni, V, Massimino, M, Lopez Aguilar, E, Torsvik, IK, Joao Gil-da-Costa, M, Kumirova, E, Cruz-Martinez, O, Holm, S, Bailey, S, Hayden, T, Thomale, UW, Janssens, GOR, Kramm, CM & van Vuurden, DG 2019, 'Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?' Journal of Neuro-Oncology, vol. 145, no. 1, pp. 177-184. https://doi.org/10.1007/s11060-019-03287-9, https://doi.org/10.1007/s11060-019-03287-9

Diagnostics and treatment of diffuse intrinsic pontine glioma : where do we stand? / el-Khouly, Fatma E.; Veldhuijzen van Zanten, Sophie E. M.; Santa-Maria Lopez, Vicente; Hendrikse, N. Harry; Kaspers, Gertjan J. L.; Loizos, G.; Sumerauer, David; Nysom, Karsten; Pruunsild, Kaie; Pentikainen, Virve; Thorarinsdottir, Halldora K.; Rutkauskiene, Giedre; Calvagna, Victor; Drogosiewicz, Monika; Dragomir, Monica; Deak, Ladislav; Kitanovski, Lidija; von Bueren, Andre O.; Kebudi, Rejin; Slavc, Irene; Jacobs, Sandra; Jadrijevic-Cvrlje, Filip; Entz-Werle, Natacha; Grill, Jacques; Kattamis, Antonis; Hauser, Peter; Pears, Jane; Biassoni, Veronica; Massimino, Maura; Lopez Aguilar, Enrique; Torsvik, Ingrid K.; Joao Gil-da-Costa, Maria; Kumirova, Ella; Cruz-Martinez, Ofelia; Holm, Stefan; Bailey, Simon; Hayden, Tim; Thomale, Ulrich W.; Janssens, Geert O. R.; Kramm, Christof M.; van Vuurden, Dannis G.

In: Journal of Neuro-Oncology, Vol. 145, No. 1, 2019, p. 177-184.

Research output: Contribution to journalArticleAcademicpeer-review

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

Y1 - 2019

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.

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UR - https://www.ncbi.nlm.nih.gov/pubmed/31522324

U2 - 10.1007/s11060-019-03287-9

DO - 10.1007/s11060-019-03287-9

M3 - Article

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SP - 177

EP - 184

JO - Journal of Neuro-Oncology

JF - Journal of Neuro-Oncology

SN - 0167-594X

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ER -