Frazier James L, Lee James, Thomale Ulrich W, Noggle Joseph C, Cohen Kenneth J, Jallo George I
Departments of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Neurosurg Pediatr. 2009 Apr;3(4):259-69. doi: 10.3171/2008.11.PEDS08281.
Diffuse intrinsic pontine gliomas constitute ~ 60-75% of tumors found within the pediatric brainstem. These malignant lesions present with rapidly progressive symptoms such as cranial nerve, long tract, or cerebellar dysfunctions. Magnetic resonance imaging is usually sufficient to establish the diagnosis and obviates the need for surgical biopsy in most cases. The prognosis of the disease is dismal, and the median survival is < 12 months. Resection is not a viable option. Standard therapy involves radiotherapy, which produces transient neurological improvement with a progression-free survival benefit, but provides no improvement in overall survival. Clinical trials have been conducted to assess the efficacy of chemotherapeutic and biological agents in the treatment of diffuse pontine gliomas. In this review, the authors discuss recent studies in which systemic therapy was administered prior to, concomitantly with, or after radiotherapy. For future perspective, the discussion includes a rationale for stereotactic biopsies as well as possible therapeutic options of local chemotherapy in these lesions.
弥漫性脑桥内生型胶质瘤约占小儿脑干肿瘤的60 - 75%。这些恶性病变表现为迅速进展的症状，如颅神经、长束或小脑功能障碍。磁共振成像通常足以确诊，在大多数情况下无需进行手术活检。该疾病预后不佳，中位生存期<12个月。手术切除不是可行的选择。标准治疗包括放疗，放疗可产生短暂的神经功能改善并带来无进展生存期获益，但对总生存期无改善。已开展临床试验评估化疗药物和生物制剂治疗弥漫性脑桥胶质瘤的疗效。在本综述中，作者讨论了近期在放疗前、放疗期间或放疗后进行全身治疗的研究。展望未来，讨论内容包括立体定向活检的理论依据以及这些病变局部化疗的可能治疗选择。
