MPC-16 RAPID PROGRESSIVE SPINAL DIFFUSE MIDLINE GLIOMA A CASE REPORT

摘要

A 17-year-old boy presented with a 2-week history of lower back pain, progressive gait difficulty and sensory deficit of bilateral lower limbs. Magnetic resonance imaging of neuroaxis showed intramedullary tumor with spinal cord expansion from Th12 to L2 and irregular areas of enhancement. Emergent laminoplasty and biopsy was performed. Histopathological examination showed small atypical cells, but most cells had too much degeneration and necrosis to confirm the diagnosis definitively. Leptomeningeal dissemination caused conscious disturbance, nuchal rigidity and epilepsy. 2 weeks after decompression, we performed cordotomy again for advanced diagnosis, to be found diffuse midline glioma, H3K27M mutant by immunohistopathological examination and DAN sequence. He was treated with combination of whole brain and spine radiation therapy and chemotherapy with temozolomide and bevacizumab. He is still alive over 6 months. The clinical significance of H3K27M mutant in spinal gliomas is unclear. Further examinations are needed.