DIPG-31. CLINICO-PATHOLOGICAL REVIEW OF 26 CHILDREN WITH DIFFUSE INTRINSIC PONTINE GLIOMA

• Published in: Neuro-oncology (Charlottesville, Va.) Va.), 2017-06, Vol.19 (suppl_4), p.iv11-iv12
• Main Authors: Terashima, Keita, Yoshida, Kaoru, Kiyotani, Chikako, Shioda, Yoko, Osumi, Tomoo, Kato, Motohiro, Tomizawa, Daisuke, Usami, Kenichi, Ogiwara, Hideki, Tsutsumi, Yoshiyuki, Fuji, Hiroshi, Yoshioka, Takako, Iwafuchi, Hideto, Nakano, Yoshiko, Ichimura, Koichi, Matsumoto, Kimikazu
• Format: Article
• Language: English
• Subjects: Abstracts
• ISSN: 1522-8517; 1523-5866
• DOI: 10.1093/neuonc/nox083.046

METHODS: Twenty-six patients with diffuse intrinsic pontine glioma (DIPG) were retrospectively analyzed at National Center for Child Health and Development, one of the largest pediatric brain tumor centers in Japan. The following information was collected from the electric medical record: clinical characteristics, surgery / pathology reports, treatments and outcomes. RESULTS: Between June, 2002 and April, 2016, 26 patients were diagnosed as DIPG and treated. Median age was 6 years (2–12). There were 19 boys and 7 girls. Common presenting symptoms included, cranial nerve palsies, ataxia, long-tract signs, vomiting, and altered consciousness. 20 patients (77%) were diagnosed within 40 days from symptom onset. 13 patients (50%) underwent biopsy. Patients who underwent biopsy tend to have atypical imaging and clinical features. Interval from diagnosis to radiation therapy was significantly longer in biopsy group than non-biopsy group (29 days and 8 days respectively, p=0.04). Histopathlogy diagnoses were: glioblastoma 4, anaplastic astrocytoma 5 and other glioma 4. H3 K27M mutations were detected in 5 patients among 8 patients tested (63%). CMV protein was not detected in all 4 patients tested. All but one patient underwent focal radiation therapy (RT) of 54Gy/30 fractions and one patient presented with dissemination underwent craniospinal RT. Fourteen patients (54%) did not receive any chemotherapy as an upfront treatment. Seven patients (27%) received temozolomide (TMZ)-based chemotherapy and the rest (19%) received other chemotherapy regimen. Mean overall survival (OS) of all 26 patients was 313 days. Mean progression-free survival (PFS) was 201 days. Biopsy, TMZ, any chemotherapy or H3 K27M mutation was not associated with survival outcomes. CONCLUSIONS: DIPG continues to be an incurable disease. Biopsy in DIPG is safe and feasible. H3 K27M mutation is a frequent event in DIPG. Chemotherapy is not a standard upfront treatment for DIPG.