DIPG-20. DIFFUSE INTRINSIC PONTINE GLIOMA IN CHILDREN. RESULTS OF ANALYSES IN THE MULTICENTER RETROSPECTIVE STUDY

BACKGROUND/OBJECTIVES. We present the results of multicenter retrospective study in children with diffuse intrinsic pontine glioma (DIPG). DESIGN/METHODS. From January 2010 to October 2018 142 patients with DIPG were observed/treated in Rogachev’s center from 16 regional hospitals of Russia. All details of follow-up were available in 57 pts (40%). MRI was the main confirmation of diagnosis, 1 pt was biopsied, 2 – received partial resection. Histological types: 1 - DIPG, 1 - HGG, in 1 patient the biopsy wasn’t informative. The distribution on the age in 57 pts (median 60 mths, 17–180 m): 9 (15,8%) patients was younger than 3 years, 48 (84,2%) patients were older than 3 years. There were 29 (50.8%) girls and 28 (49.1%) boys. 48 patients (84%) who received RT/CHT, and 9 (15,7%) patients were not treated. RT was performed in 47 patients, among them 20 (42%) – with parallel Temozolomid (TMZ), in 1 - with Valproic acid. 27 patients (47%) showed positive response to the therapy. 16 patients (28%) received TMZ after radiation therapy, 8 (50%) of them had positive answer. 19 pts (33%) are alive. The median to the first progression after RT was 9 m (1–57 m). The median of OS 12 m (1–63 m). Among 20 pts, who received TMZ during RT 17 pts died (85%). Among 26 pts, who did not receive TMZ 15 pts died (57,7%). Salvage (second) RT was performed in 17 children (29%) among them only 2 pts (11,8%) are alive. CONCLUSIONS: It is well know that not any therapy of DIPG can provide long-term survival. Adding TMZ to RT and salvage RT does not improve the effect of primary and relapse treatment also in our cohort. Further research is needed in this area for the development of new treatment strategies.