Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.
Am J Clin Oncol. 2013 Jun;36(3):287-92. doi: 10.1097/COC.0b013e3182467efa.
To evaluate the clinical characteristics and long-term outcome of pediatric patients with optic glioma.
A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease.
The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease.
Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies.
评估儿童视神经胶质瘤患者的临床特征和长期预后。
回顾性评估了 1975 年至 2008 年间新诊断的 101 例视神经胶质瘤患者。COPP（环磷酰胺、长春新碱、洛莫司汀、泼尼松）和顺铂联合依托泊苷是最常用的化疗方案。对进展性或无反应性疾病的患者进行放疗。
诊断时的中位年龄为 6 岁，男女比例为 1.15。最常见的转诊主诉是斜视。最常见的视神经胶质瘤部位是下丘脑-视交叉区（31.7%）。53 例（52.5%）患者患有神经纤维瘤病 1 型（NF-1）。治疗包括手术、放疗和化疗。49 例（48.5%）患者接受手术治疗，主要为次全切除术，39.4%患者接受放疗，30 例患者接受化疗。5 年无进展生存率（PFS）和总生存率（OS）分别为 65.8%和 88.4%，10 年 PFS 和 OS 分别为 54.2%和 83.4%，中位随访时间为 8 年。下丘脑-视交叉受累患者的 OS 明显较低，NF-1 患者的 OS 明显较高。诊断时年龄在 10 岁或以上的患者（P=0.0001）和眶内受累的患者（P=0.032）5 年和 10 年 PFS 率显著较高。18 例患者（17.8%）死于疾病。
患有 NF-1 的患者和年龄在 10 岁以上的患者预后较好，而年龄在 3 岁以下的患者和下丘脑-视交叉视神经胶质瘤患者预后较差。需要进一步研究以找到合适的治疗策略。
