Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana, USA.
J Neurosurg. 2012 Nov;117(5):825-30. doi: 10.3171/2012.7.JNS111260. Epub 2012 Sep 7.
Gangliogliomas comprise less than 1% of all brain tumors and occur most often in children. Therefore, there are a limited number of patients and data involving the use or role of adjuvant therapy after subtotal resections (STRs) of gangliogliomas. The objective of this study was to examine and review the Mayo Clinic experience of 88 patients with gangliogliomas, their follow-up, risk of recurrence, and the role of radiation therapy after STR or only biopsy.
Eighty-eight patients with gangliogliomas diagnosed between 1970 and 2007 were reviewed. Data on clinical outcomes and therapy received were analyzed. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival.
The median age at diagnosis was 19 years. The median potential follow-up as of June 2008 was 142 months (range 9-416 months). Fifteen-year overall survival was 94%, median PFS was 5.6 years, with a 10-year PFS rate of 37%. Progression-free survival was dramatically affected by extent of initial resection (p < 0.0001).
This single-institution retrospective series of patients with gangliogliomas is unique given its large cohort size with a long follow-up duration, and confirms the excellent long-term survival rate in this group. The study also shows the importance of resection extent on likelihood of recurrence. Patients with gangliogliomas who undergo STR or biopsy alone have poor PFS. Radiation therapy may delay time to progression in patients with unresectable disease.
神经节细胞瘤占所有脑肿瘤的不到 1%，最常发生在儿童中。因此，涉及神经节细胞瘤次全切除术后（STR）辅助治疗的使用或作用的患者和数据数量有限。本研究的目的是检查和回顾梅奥诊所 88 例神经节细胞瘤患者的经验、他们的随访、复发风险以及 STR 或仅活检后放射治疗的作用。
回顾了 1970 年至 2007 年间诊断的 88 例神经节细胞瘤患者。分析了临床结果和治疗数据。采用 Kaplan-Meier 法估计无进展生存率（PFS）和总生存率。
诊断时的中位年龄为 19 岁。截至 2008 年 6 月的中位潜在随访时间为 142 个月（范围 9-416 个月）。15 年总生存率为 94%，中位 PFS 为 5.6 年，10 年 PFS 率为 37%。无进展生存率明显受初始切除范围的影响（p<0.0001）。
由于该队列规模大、随访时间长，本机构回顾性系列研究是独特的，证实了该组患者的长期生存率非常优异。该研究还表明切除范围对复发可能性的重要性。仅接受 STR 或活检的神经节细胞瘤患者 PFS 较差。放射治疗可能会延迟无法切除疾病患者的进展时间。
