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Eur J Cancer. 2017 Mar;73:38-47. doi: 10.1016/j.ejca.2016.12.007. Epub 2017 Feb 3.

Survival benefit for patients with diffuse intrinsic pontine glioma (DIPG) undergoing re-irradiation at first progression: A matched-cohort analysis on behalf of the SIOP-E-HGG/DIPG working group.

Author information

1
Department of Radiation Oncology, University Medical Center Utrecht and Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands. Electronic address: g.o.r.janssens@umcutrecht.nl.
2
Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
3
Department of Radiotherapy, Gustave Roussy Cancer Campus, Villejuif Cedex, France.
4
Department of Clinical Oncology, The Royal Marsden NHS Foundation Trust, Sutton, UK.
5
Department of Radiation Oncology, Hospital Vall d'Hebron, Barcelona, Spain.
6
Department of Radiation Oncology, Leuven Cancer Institute, University Hospitals Leuven, Leuven, Belgium.
7
Department of Clinical Oncology, University Hospital Birmingham, Birmingham, UK.
8
Department of Radiation Oncology, Radboud University Medical Centre, Nijmegen, The Netherlands.
9
Department of Pediatric Hematology and Oncology, Hospital Sant Joan de Deu, Barcelona, Spain.
10
Department of Radiation Therapy, University Hospital Leipzig, Leipzig, Germany.
11
Pediatric Oncology Unit, Great Ormond Street Hospital, London, UK.
12
Pediatrics Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
13
Department of Hematology & Oncology, University of Geneva, Geneva, Switzerland; Department of Pediatric Hematology & Oncology, University Hospital Goettingen, Goettingen, Germany.
14
Department of Pediatric Oncology & Hematology, VU University Medical Center, Amsterdam, The Netherlands.
15
Division of Pediatric Neuro-oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
16
Department of Radiation Oncology, University Medical Center Utrecht and Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands.
17
Department of Pediatric Hematology & Oncology, University Hospital Goettingen, Goettingen, Germany.

Abstract

BACKGROUND:

Overall survival (OS) of patients with diffuse intrinsic pontine glioma (DIPG) is poor. The purpose of this study is to analyse benefit and toxicity of re-irradiation at first progression.

METHODS:

At first progression, 31 children with DIPG, aged 2-16 years, underwent re-irradiation (dose 19.8-30.0 Gy) alone (n = 16) or combined with systemic therapy (n = 15). At initial presentation, all patients had typical symptoms and characteristic MRI features of DIPG, or biopsy-proven high-grade glioma. An interval of ≥3 months after upfront radiotherapy was required before re-irradiation. Thirty-nine patients fulfilling the same criteria receiving radiotherapy at diagnosis, followed by best supportive care (n = 20) or systemic therapy (n = 19) at progression but no re-irradiation, were eligible for a matched-cohort analysis.

RESULTS:

Median OS for patients undergoing re-irradiation was 13.7 months. For a similar median progression-free survival after upfront radiotherapy (8.2 versus 7.7 months; P = .58), a significant benefit in median OS (13.7 versus 10.3 months; P = .04) was observed in favour of patients undergoing re-irradiation. Survival benefit of re-irradiation increased with a longer interval between end-of-radiotherapy and first progression (3-6 months: 4.0 versus 2.7; P < .01; 6-12 months: 6.4 versus 3.3; P = .04). Clinical improvement with re-irradiation was observed in 24/31 (77%) patients. No grade 4-5 toxicity was recorded. On multivariable analysis, interval to progression (corrected hazard ratio = .27-.54; P < .01) and re-irradiation (corrected hazard ratio = .18-.22; P < .01) remained prognostic for survival. A risk score (RS), comprising 5 categories, was developed to predict survival from first progression (ROC: .79). Median survival ranges from 1.0 month (RS-1) to 6.7 months (RS-5).

CONCLUSIONS:

The majority of patients with DIPG, responding to upfront radiotherapy, do benefit of re-irradiation with acceptable tolerability.

KEYWORDS:

Diffuse intrinsic pontine glioma (DIPG); Matched-cohort analysis; Radiotherapy; Re-irradiation; Survival prediction model

PMID:
28161497
DOI:
10.1016/j.ejca.2016.12.007
[Indexed for MEDLINE]

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