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| Journal of neuro-oncology. 2019 Sep 14. doi: 10.1007/s11060-019-03287-9. pii: 10.1007/s11060-019-03287-9 |
| Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand? |
| El-Khouly FE1, Veldhuijzen van Zanten SEM2, Santa-Maria Lopez V3, Hendrikse NH4, Kaspers GJL5, Loizos G6, Sumerauer D7, Nysom K8, Pruunsild K9, Pentikainen V10, Thorarinsdottir HK11, Rutkauskiene G12, Calvagna V13, Drogosiewicz M14, Dragomir M15, Deak L16, Kitanovski L17, von Bueren AO18, Kebudi R19, Slavc I20, Jacobs S21, Jadrijevic-Cvrlje F22, Entz-Werle N23, Grill J24, Kattamis A25, Hauser P26, Pears J27, Biassoni V28, Massimino M29, Lopez Aguilar E30, Torsvik IK31, Joao Gil-da-Costa M32, Kumirova E33, Cruz-Martinez O34, Holm S35, Bailey S36, Hayden T37, Thomale UW38, Janssens GOR39, Kramm CM40, van Vuurden DG41 |
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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. |
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KEYWORDS: Chemotherapy, Diffuse intrinsic pontine glioma (DIPG), Diffuse midline glioma H3-K27 mutant (DMG K3-27M), Radiotherapy |
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Publikations ID: 31522324 Quelle: öffnen |
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