Clark Aaron J, Cage Tene A, Aranda Derick, Parsa Andrew T, Sun Peter P, Auguste Kurtis I, Gupta Nalin
Department of Neurological Surgery, Pediatric Neurosurgery, University of California, San Francisco, San Francisco, CA 94143-0112, USA.
Childs Nerv Syst. 2013 Feb;29(2):231-8. doi: 10.1007/s00381-012-1926-2. Epub 2012 Oct 23.
Craniopharyngiomas are rare tumors with bimodal incidence in the pediatric and adult age groups. Treatment strategies range from aggressive resection to planned limited resection combined with adjuvant therapies. Currently there is no consensus for standard of care for pediatric craniopharyngioma.
We performed a systematic review of the published literature on pediatric craniopharyngioma. Patients were grouped based on extent of resection into gross total resection (GTR), subtotal resection (STR), and biopsy procedures. These groups were compared with respect to tumor control. Chi square was used to compare rates of recurrence. Kaplan-Meier was used to generate progression-free survival (PFS) estimates. Cox proportional hazard modeling was used to evaluate risk of progression. Each extent of resection group was also subdivided based on adjuvant therapy and compared.
A total of 109 studies described extent of resection resulting in a cohort of 531 patients. Recurrence data were available for 377 patients. There was no difference in 1- or 5-year PFS between the groups who underwent GTR and STR combined with radiation (XRT; log-rank; p = 0.76; 1-year PFS 89 vs 84%; 5-year PFS 77 vs 73%, respectively). One-year PFS was 84% for STR+XRT compared to 76% for STR alone while 5-year PFS was 73% for STR+XRT compared to 43% for STR alone (log-rank; p = 0.003).
Although there are limitations of a systematic review of retrospective data, our results suggest that STR+XRT of pediatric craniopharyngioma is associated with similar rates of tumor control as GTR.
颅咽管瘤是一种罕见肿瘤，在儿童和成人年龄组中发病率呈双峰分布。治疗策略从积极切除到计划性有限切除联合辅助治疗不等。目前，儿童颅咽管瘤的标准治疗方案尚无共识。
我们对已发表的关于儿童颅咽管瘤的文献进行了系统综述。根据切除范围将患者分为大体全切除（GTR）、次全切除（STR）和活检手术组。比较这些组在肿瘤控制方面的情况。采用卡方检验比较复发率。使用Kaplan-Meier法生成无进展生存期（PFS）估计值。采用Cox比例风险模型评估进展风险。每个切除范围组还根据辅助治疗进行细分并比较。
共有109项研究描述了切除范围，纳入了531例患者。377例患者有复发数据。接受GTR和STR联合放疗（XRT）的组之间1年或5年PFS无差异（对数秩检验；p = 0.76；1年PFS分别为89%和84%；5年PFS分别为77%和73%）。STR+XRT组的1年PFS为84%，而单纯STR组为76%；STR+XRT组的5年PFS为73%，而单纯STR组为43%（对数秩检验；p = 0.003）。
尽管对回顾性数据进行系统综述存在局限性，但我们的结果表明，儿童颅咽管瘤的STR+XRT与GTR的肿瘤控制率相似。
