DIPG-91. CLINICAL, RADIOGRAPHIC, HISTOPATHOLOGIC, AND MOLECULAR CHARACTERISTICS OF LONG-TERM SURVIVORS OF DIFFUSE INTRINSIC PONTINE GLIOMA-AN UPDATE FROM THE INTERNATIONAL AND EUROPEAN SOCIETY FOR PEDIATRIC ONCOLOGY DIPG REGISTRIES (IDIPGR/SIOPE-DIPGR)

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Abstract BACKGROUND Diffuse intrinsic pontine glioma (DIPG) is a childhood tumor with median survival of < 1 year. Survival-associated factors for this rare disease are poorly understood. METHODS Clinical, radiological, and histo-molecular data were abstracted from databases of the IDIPGR and SIOPE-DIPGR and analyzed using descriptive statistics. Survival was estimated by the Kaplan Meier method. Short term survivors (STS) were defined as overall survival (OS) < 2 years, and long term survivors (LTS) had OS >2 years. We present data from the IDIPGR with additional analyses forthcoming. RESULTS Analysis included 1123 patients with DIPG, with 476 undergoing central radiographic review. Median OS for the entire cohort was 11 months (IQR 7-15 months), with 2 yr OS 6.9% (5.6% to 8.6%). Median age at diagnosis was 6.6 years (5-9.7 years) for 1045 STS (93.1%) and 6.8 years (3.7-13 years) for 78 LTS (6.9%). Factors significantly associated with LTS included longer symptom duration (STS 73.7% vs LTS 42%; p<0.001), age < 3 and > 10 years (STS 4.5 and 22.8% vs LTS 17.9 and 35.9%; p<0.0001), and receipt of chemotherapy at any time during therapy (STS 54.7% vs LTS 67.9%; p=0.025). A similar percentage of STS and LTS received EGFR, MTOR, PI3K, HDAC, local delivery and immunotherapy, although more LTS used VEGF inhibition (20.5 % vs 8.6%; p = 0.002). Of 104 patients with molecular data, 69 harbored the H3.3 histone mutations (7 LTS, 62 STS), and 15 had H3.1(3 LTS, 12 STS). CONCLUSION Early analysis of factors associated with survival in patients with DIPG reveal similar findings as the prior IDIPGR/SIOPE-DIPGR study in 2018. These findings will be updated for the final presentation to include ~400 more cases and additional radiological and molecular data.

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