10249-PEDT-8 CURRENT STATUS AND ISSUES OF PEDIATRIC DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)

Abstract

Abstract that predominantly occurs in childhood, with a median survival of less than one year. Herein we present a recent case of DIPG and discuss the current status and future challenges. The patient was a 7-year-old boy who complained of tinnitus, headaches, and unstable gait. The MRI showed diffuse enlargement of the pons, and the contrast-enhanced T1-weighted image exhibited an irregular ring-like enhancement. Neurologically, he presented with left abducens nerve palsy, horizontal gaze nystagmus, significant left ataxia, and truncal ataxia. As the lesion extended to the left cerebellar peduncle, a stereotactic biopsy was performed. However, postoperative bleeding occurred, resulting in transient consciousness disturbance, left facial palsy, speech disturbance, and dysphagia, necessitating enteral nutrition management. The pathological diagnosis was high-grade glioma, IDH wild type, H3K27M mutation (-), TERTp wild type, BRAF V600E mutation (-), BRAF fusion (-), and MGMT unmethylated. After surgery, the patient received radiation therapy (54Gy/30fr), and his symptoms improved, allowing him to resume oral intake. Post-irradiation, the lesion shrank, and he was discharged home without assistance. However, six months later, his symptoms worsened, and he started taking temozolomide orally, but the effect was limited. Nine months later, he became unable to take oral medication, and a central venous port was placed. We coordinated with home care physicians to provide intravenous and palliative treatments, but respiratory and consciousness disturbances gradually worsened, and he died 10 months after the initial diagnosis. The median survival of DIPG patients treated at our hospital is currently 10.5 months, and this case followed a typical course. The development of novel treatments is desired, but due to the rarity of the disease, conducting comparative trials is challenging. Hence, registry data forming a control group are necessary. Additionally, transitioning to home-based palliative care and sharing information on supportive therapies are also critical tasks.