Faculty of Medicine, Université Laval.
Division of Radiation Oncology.
J Pediatr Hematol Oncol. 2024 May 1;46(4):211-215. doi: 10.1097/MPH.0000000000002853. Epub 2024 Apr 4.
Diffuse intrinsic pontine gliomas are lethal tumors with a prognosis generally less than 1 year. Few cases of survivors of 5 years or more have been reported. This case report highlights the journey of a 9.5-year survivor who underwent 3 rounds of focal radiotherapy; she experienced 6 years of progression-free survival following the first round but ultimately succumbed to her disease. An autopsy revealed a favorable IDH1 mutation and the absence of H3K27M. This case reiterates the importance of extensive molecular analyses in diffuse intrinsic pontine gliomas and explores the potential benefit of re-irradiation in patients with positive responses and long periods of remission.
弥漫性内生脑桥胶质瘤是致命的肿瘤，一般预后不到 1 年。报告的 5 年以上幸存者病例很少。本病例报告强调了一名 9.5 年幸存者的历程，她接受了 3 轮局部放疗；在第一轮治疗后，她经历了 6 年的无进展生存期，但最终还是死于疾病。尸检显示 IDH1 突变良好，H3K27M 缺失。本病例再次强调了在弥漫性内生脑桥胶质瘤中进行广泛分子分析的重要性，并探讨了对有阳性反应和长时间缓解的患者进行再放疗的潜在益处。
