Journal
JOURNAL OF CLINICAL ONCOLOGY
Volume 36, Issue 19, Pages 1963-+Publisher
AMER SOC CLINICAL ONCOLOGY
DOI: 10.1200/JCO.2017.75.9308
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Funding
- Diffuse Intrinsic Pontine Glioma (DIPG) Collaborative
- Cure Starts Now Foundation
- Hope for Caroline Foundation
- Julian Boivin Courage for Cures Foundation
- Abbie's Army
- Michael Mosier Defeat DIPG Foundation
- Reflections of Grace Foundation
- Cure Starts Now Australia
- Brooke Healey Foundation
- Soar With Grace Foundation
- Jeffrey Thomas Hayden Foundation
- Cure Brain Cancer Foundation
- Jones Family Foundation
- Musella Foundation
- Pray, Hope Believe Foundation
- Smiles for Sophie Foundation
- Benny's World
- Love Chloe Foundation
- Aiden's Avengers
- A Cure from Caleb Society
- Operation Grace White Foundation
- Ryan's Hope
- Wayland Villars DIPG Foundation
- American Childhood Cancer Organization
- Juliana Rose Donnelly Trust
- Sheila Jones and Friends
- Ellie Kavalieros DIPG Research Fund
- Voices Against Brain Cancer
- Semmy Foundation
- Associazione Bianca Garavaglia Onlus
- Il fondo di Gio
- Vrije Universiteit Medical Center CCA Children
- Deutsche Kinderkrebsstiftung
- NATIONAL CANCER INSTITUTE [ZIABC011447, ZIABC011298] Funding Source: NIH RePORTER
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PurposeDiffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and histomolecular characteristics between short-term survivors (STSs) and long-term survivors (LTSs).Materials and MethodsData abstracted from registry databases included patients from North America, Australia, Germany, Austria, Switzerland, the Netherlands, Italy, France, the United Kingdom, and Croatia.ResultsAmong 1,130 pediatric and young adults with radiographically confirmed DIPG, 122 (11%) were excluded. Of the 1,008 remaining patients, 101 (10%) were LTSs (survival 2 years). Median survival time was 11 months (interquartile range, 7.5 to 16 months), and 1-, 2-, 3-, 4-, and 5-year survival rates were 42.3% (95% CI, 38.1% to 44.1%), 9.6% (95% CI, 7.8% to 11.3%), 4.3% (95% CI, 3.2% to 5.8%), 3.2% (95% CI, 2.4% to 4.6%), and 2.2% (95% CI, 1.4% to 3.4%), respectively. LTSs, compared with STSs, more commonly presented at age < 3 or > 10 years (11% v 3% and 33% v 23%, respectively; P < .001) and with longer symptom duration (P < .001). STSs, compared with LTSs, more commonly presented with cranial nerve palsy (83% v 73%, respectively; P = .008), ring enhancement (38% v 23%, respectively; P = .007), necrosis (42% v 26%, respectively; P = .009), and extrapontine extension (92% v 86%, respectively; P = .04). LTSs more commonly received systemic therapy at diagnosis (88% v 75% for STSs; P = .005). Biopsies and autopsies were performed in 299 patients (30%) and 77 patients (10%), respectively; 181 tumors (48%) were molecularly characterized. LTSs were more likely to harbor a HIST1H3B mutation (odds ratio, 1.28; 95% CI, 1.1 to 1.5; P = .002).ConclusionWe report clinical, radiologic, and molecular factors that correlate with survival in children and young adults with DIPG, which are important for risk stratification in future clinical trials.
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