Cage Tene A, Clark Aaron J, Aranda Derick, Gupta Nalin, Sun Peter P, Parsa Andrew T, Auguste Kurtis I
Department of Neurological Surgery, University of California-San Francisco, CA 94143, USA.
J Neurosurg Pediatr. 2013 Jun;11(6):673-81. doi: 10.3171/2013.2.PEDS12345. Epub 2013 Mar 29.
Ependymoma is the third most common primary brain tumor in children. Tumors are classified according to the WHO pathological grading system. Prior studies have shown high levels of variability in patient outcomes within and across pathological grades. The authors reviewed the results from the published literature on intracranial ependymomas in children to describe clinical outcomes as they relate to treatment modality, associated mortality, and associated progression-free survival (PFS).
A search of English language peer-reviewed articles describing patients 18 years of age or younger with intracranial ependymomas yielded data on 182 patients. These patients had undergone treatment for ependymoma with 1 of 5 modalities: 1) gross-total resection (GTR), 2) GTR as well as external beam radiation therapy (EBRT), 3) subtotal resection (STR), 4) STR as well as EBRT, or 5) radiosurgery. Mortality and outcome data were analyzed for time to tumor progression in patients treated with 1 of these 5 treatment modalities.
Of these 182 patients, 69% had supratentorial ependymomas and 31% presented with infratentorial lesions. Regardless of tumor location or pathological grade, STR was associated with the highest rates of mortality. In contrast, GTR was associated with the lowest rates of mortality, the best overall survival, and the longest PFS. Children with WHO Grade II ependymomas had lower mortality rates when treated more aggressively with GTR. However, patients with WHO Grade III tumors had slightly better survival outcomes after a less aggressive surgical debulking (STR+EBRT) when compared with GTR.
Mortality, PFS, and overall survival vary in pediatric patients with intracranial ependymomas. Pathological classification, tumor location, and method of treatment play a role in outcomes. In this study, GTR was associated with the best overall and PFS rates. Patients with WHO Grade II tumors had better overall survival after GTR+EBRT and better PFS after GTR alone. Patients with WHO Grade III tumors had better overall survival after STR+EBRT. Patients with infratentorial tumors had improved overall survival compared with those with supratentorial tumors. Progression-free survival was best in those patients with infratentorial tumors following STR+EBRT. Consideration of all of these factors is important when counseling families on treatment options.
室管膜瘤是儿童中第三常见的原发性脑肿瘤。肿瘤根据世界卫生组织（WHO）病理分级系统进行分类。先前的研究表明，在不同病理分级内及不同分级之间，患者的预后存在高度变异性。作者回顾了已发表的关于儿童颅内室管膜瘤的文献结果，以描述与治疗方式、相关死亡率及无进展生存期（PFS）相关的临床预后。
检索描述18岁及以下颅内室管膜瘤患者的英文同行评议文章，获得了182例患者的数据。这些患者接受了以下5种治疗方式之一来治疗室管膜瘤：1）全切除（GTR）；2）全切除以及外照射放疗（EBRT）；3）次全切除（STR）；4）次全切除以及EBRT；或5）立体定向放射外科治疗。分析了接受这5种治疗方式之一的患者的死亡率及肿瘤进展时间的预后数据。
在这182例患者中，69%患有幕上室管膜瘤，31%表现为幕下病变。无论肿瘤位置或病理分级如何，次全切除的死亡率最高。相比之下，全切除的死亡率最低、总体生存率最佳且无进展生存期最长。WHO二级室管膜瘤患儿接受更积极的全切除治疗时死亡率较低。然而，与全切除相比，WHO三级肿瘤患者在接受不太积极的手术减瘤（次全切除+EBRT）后生存结果略好。
儿童颅内室管膜瘤患者的死亡率、无进展生存期和总体生存率各不相同。病理分类、肿瘤位置和治疗方法对预后有影响。在本研究中，全切除与最佳的总体生存率和无进展生存率相关。WHO二级肿瘤患者在全切除+EBRT后总体生存率更佳，单独全切除后无进展生存期更佳。WHO三级肿瘤患者在次全切除+EBRT后总体生存率更佳。幕下肿瘤患者的总体生存率高于幕上肿瘤患者。幕下肿瘤患者在次全切除+EBRT后的无进展生存期最佳。在为家庭提供治疗选择咨询时，考虑所有这些因素很重要。
