Abstract:
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.
摘要:
弥漫性内源性脑桥胶质瘤是预后通常小于1年的致死性肿瘤。很少报道5年或更长时间的幸存者病例。此病例报告重点介绍了一个9.5年的幸存者的旅程,她接受了3轮局灶性放疗;她经历了6年的无进展生存后第一轮,但最终死于疾病。尸检显示有利的IDH1突变和H3K27M的缺失。此病例重申了广泛分子分析在弥漫性脑桥脑胶质瘤中的重要性,并探讨了对反应积极且长期缓解的患者进行再次照射的潜在益处。