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

• Published in: Journal of neuro-oncology 2019-10, Vol.145 (1), p.177-184
• Main Authors: 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.
• Format: Article
• Language: English
• Subjects: Biopsy; Brain cancer; Brain stem; Brain Stem Neoplasms - diagnosis; Brain Stem Neoplasms - therapy; Chemotherapy; Children; Clinical Study; Clinical trials; Combined Modality Therapy; Diagnosis; Diffuse Intrinsic Pontine Glioma - diagnosis; Diffuse Intrinsic Pontine Glioma - therapy; Disease Progression; Glioma; Humans; Life Sciences; Malignancy; Medicine; Medicine & Public Health; Neurology; Oncology; Patients; Prognosis; Radiation therapy
• ISSN: 0167-594X; 1573-7373
• DOI: 10.1007/s11060-019-03287-9

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.