弥漫性内生性脑桥胶质瘤基础与临床研究进展

Basic and clinical research progress of diffuse intrinsic pontine glioma

弥漫性内生性脑桥胶质瘤（DIPG）是一种发生在脑干的高度侵袭性神经胶质瘤，在儿童期发病，约占儿科脑及中枢神经系统肿瘤发病率的10%～15%。DIPG 预后极差，仅有不到10%的患儿生存期超过2年。DIPG 的影像学表现非常典型，活检仅在非典型病例实施，治疗前的组织标本很少，限制了该病的分子生物学研究。近年来手术和分子分析技术的进展突飞猛进，活检的安全性大大提高，脑干活检的应用逐步纳入许多临床试验中，DIPG 的分子病理学研究及治疗将取得新的突破。

Diffuse intrinsic pontine glioma（ DIPG）is a highly invasive tumor located in the pons （middle）of the brain stem. They are usually diagnosed during childhood and account for 10% -15% of primary brain tumors in children. DIPG has a very poor prognosis. Fewer than 10% of DIPG patients survive more than 2 years after diagnosis. The imaging manifestations of DIPG are typical,and biopsy is only performed in atypi-cal cases. The tissue specimens of newly diagnosed DIPG are very few and limit its molecular biological research. Recent advances in surgical and molecular-analytic techniques have increased the safety of biopsy which has already been used in many clinical trials step by step. The research of DIPG′s molecular pathogenesis and treatment is sure to achieve new breakthroughs.