9567 Background: The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. Despite various studies undertaken to improve outcome, radiotherapy (RT) remains the standard treatment, which is mostly palliative. This study aims to evaluate characteristics and treatment outcome of children with DIPG in a single center. Methods: We retrospectively reviewed the demographic, clinical characteristics and treatment outcome of children with DIPG treated at Istanbul University, Oncology Institute from 1999 to 2011. We also evaluated the group that prospectively recieved RT with concurrent and adjuvant temozolamide after 2004. Results: 47 children (24 female, 23 male) with the median age of 7 years (6 months-16 years) were analyzed. The median duration of symptoms was 30 days (2-630 days). The frequent clinical findings were ataxia, strabismus and motor weakness. All patients received RT, 54-60 Gy to the tumor site. 12 recieved only RT. 35 had concomitant and/or adjuvant chemotherapy with RT. 8 recieved cisplatinum, 7 vincristine. Since 2004, 20 patients recieved the institutional protocol consisting of temozolomide (TMZ) (75 mg/m2/day) for 6 weeks concurrent with RT, followed by TMZ (200 mg/m2/day) for 5 days every 28 days for 12 cycles or until progression. There was no major side effect due to TMZ, thrombocytopenia being the most frequent, but managable side effect. The median overall survival after diagnosis was 13 months (3-132 mo.) for the whole group. The median overall survival in 20 patients that received RT and TMZ [ 17 months (3-132 months)], was significantly superior than that in 12 patients that recieved only RT [ 12 months (3-20 months)] ( (p=0.03). Nimotuzumab was given to 4 patients that progressed after RT and TMZ. There was no major side effect due to nimotuzumab. One was stable for 1 year with significant clinical improvement, the others were stable for 5, 2 and 2 months after nimotuzumab. Conclusions: In our series, the median survival was significantly superior in patients who received RT with concurrent and adjuvant temozolamide in comparison to patients that recieved RT alone. Nimotuzumab may be promising in some progressive patients, its role as upfront treatment needs further investigation.
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