Diffuse intrinsic pontine glioma (DIPG) is a rare but extremely malignant central nervous system tumor primarily affecting children that is almost universally fatal with a devastating prognosis of 8-to-12-month median survival time following diagnosis. Traditionally, DIPG has been diagnosed via MR imaging alone and treated with palliative radiation therapy. While performing surgical biopsies for these patients has been controversial, in recent years, advancements have been made in the safety and efficacy of surgical biopsy techniques, utilizing stereotactic, robotics, and intraoperative cranial nerve monitoring as well as the development of liquid biopsies that identify tumor markers in either cerebrospinal fluid or serum. With more molecular data being collected from these tumors due to more frequent biopsies being performed, multiple treatment modalities including chemotherapy, radiation therapy, immunotherapy, and epigenetic modifying agents continue to be developed. Numerous recent clinical trials have been completed or are currently ongoing that have shown promise in extending survival for patients with DIPG. Focused ultrasound (FUS) has also emerged as an additional promising adjunct invention used to increase the effectiveness of therapeutic agents. In this review, we discuss the current evidence to date for these advancements in the diagnosis and treatment of DIPG.
弥漫性内生型脑桥胶质瘤（DIPG）是一种罕见但恶性程度极高的中枢神经系统肿瘤，主要影响儿童群体，其预后极差，确诊后中位生存期仅为8至12个月，几乎均致命。传统上，DIPG仅通过磁共振成像进行诊断，并采用姑息性放射治疗。尽管对这类患者进行手术活检一直存在争议，但近年来，手术活检技术的安全性和有效性已取得显著进展，包括采用立体定向、机器人辅助及术中颅神经监测技术，同时基于脑脊液或血清中肿瘤标志物检测的液体活检技术也得到发展。随着活检实施频率增加，从这些肿瘤中收集的分子数据日益丰富，多种治疗模式——包括化疗、放疗、免疫治疗及表观遗传修饰药物——持续得到开发。近期已完成或正在进行的多项临床试验已显示出延长DIPG患者生存期的潜力。聚焦超声（FUS）作为一种新兴的辅助治疗手段，在提升治疗药物有效性方面展现出应用前景。本文综述将系统探讨当前DIPG诊疗领域这些进展的最新证据。
Updates in Diagnostic Techniques and Experimental Therapies for Diffuse Intrinsic Pontine Glioma
肿瘤(癌症)患者之家