Department of System Cancer Science, National Cancer Center Graduate School of Cancer Science and Policy, Goyang, Republic of Korea; Center for Pediatric Cancer, National Cancer Center, Goyang, Republic of Korea.
Department of System Cancer Science, National Cancer Center Graduate School of Cancer Science and Policy, Goyang, Republic of Korea; Center for Pediatric Cancer, National Cancer Center, Goyang, Republic of Korea.
Crit Rev Oncol Hematol. 2017 Dec;120:111-119. doi: 10.1016/j.critrevonc.2017.10.013. Epub 2017 Oct 31.
Prognosis of diffuse intrinsic pontine glioma (DIPG) is poor, with a median survival of 10 months after radiation. At present, chemotherapy has failed to show benefits over radiation. Advances in biotechnology have enabled the use of autopsy specimens for genomic analyses and molecular profiling of DIPG, which are quite different from those of supratentorial high grade glioma. Recently, combined treatments of cytotoxic agents with target inhibitors, based on biopsied tissue, are being examined in on-going trials. Spontaneous DIPG mice models have been recently developed that is useful for preclinical studies. Finally, the convection-enhanced delivery could be used to infuse drugs directly into the brainstem parenchyma, to which conventional systemic administration fails to achieve effective concentration. The WHO glioma classification defines a diffuse midline glioma with a H3-K27M-mutation, and we expect increase of tissue confirmation of DIPG, which will give us the biological information helping the development of a targeted therapy.
弥漫性内在脑桥神经胶质瘤（DIPG）的预后较差，放疗后中位生存期为 10 个月。目前，化疗并未显示优于放疗的效果。生物技术的进步使得使用尸检标本进行 DIPG 的基因组分析和分子特征分析成为可能，而这些分析与幕上高级别胶质瘤有很大的不同。最近，基于活检组织，正在对细胞毒性药物与靶向抑制剂的联合治疗进行临床试验。最近还开发了自发性 DIPG 小鼠模型，可用于临床前研究。最后，通过对流增强递送，可以将药物直接注入脑实质，而常规的全身给药无法达到有效的浓度。世界卫生组织（WHO）的胶质瘤分类定义了一种弥漫性中线胶质瘤，伴有 H3-K27M 突变，我们预计 DIPG 的组织确认数量会增加，这将为我们提供有助于靶向治疗开发的生物学信息。
