Jaing Tang-Her, Lin Kuang-Lin, Tsay Pei-Kwei, Hsueh Chuen, Hung Po-Cheng, Wu Chieh-Tsai, Tseng Chen-Kan
Division of Hematology and Oncology, Department of Pediatrics, Chang Gung Children's , Chang Gung University, Taoyuan, Taiwan.
J Pediatr Hematol Oncol. 2008 Mar;30(3):222-4. doi: 10.1097/MPH.0b013e318162bd6a.
The aim of this study was to present our 17-year experience (1989 to 2006) in the treatment of optic pathway/hypothalamic gliomas (OPHG) in 18 children younger than 17 years (median age, 66 mo). Only 2 of these had evidence of neurofibromatosis-1. OPHG was diagnosed using computed tomography and/or magnetic resonance imaging. Histologic studies showed low-grade astrocytoma (WHO grade I or II) in 16 cases, anaplastic astrocytoma in 1, and oligoastrocytoma (WHO grade III) in 1. Treatment included partial tumor resection in 12 patients, chemotherapy in 5, and radiotherapy in 3. Ophthalmologic and visual alterations occurred in 12 patients, endocrine alterations in 6, and neurologic signs in 5. All treatment modalities led to tumor shrinkage and stabilization for a variable period, but none of them totally eradicated the tumor. Fourteen (78%) of 18 patients had a sustained reduction of tumor size between 6 months and 17 years. The 5-year overall and progression-free survival rates were 80.0% and 63.3%, respectively. Fifty-six percent of patients had endocrinologic sequelae, with growth hormone deficiency being the most common. Two patients died, none with neurofibromatosis-1, with a hypothalamic/chiasmatic tumor with suprasellar extension and accompanying electrolyte abnormalities. Because progression of these tumors is slow and associated with endocrinopathy, we recommend chemotherapy as a primary treatment of OPHG if the disease progresses.
本研究的目的是介绍我们在1989年至2006年期间对18名17岁以下儿童（中位年龄66个月）的视路/下丘脑胶质瘤（OPHG）的治疗经验。其中只有2名有神经纤维瘤病1型的证据。OPHG通过计算机断层扫描和/或磁共振成像进行诊断。组织学研究显示，16例为低级别星形细胞瘤（世界卫生组织I级或II级），1例为间变性星形细胞瘤，1例为少突星形细胞瘤（世界卫生组织III级）。治疗包括12例患者的部分肿瘤切除术、5例患者的化疗和3例患者的放疗。12例患者出现眼科和视觉改变，6例出现内分泌改变，5例出现神经系统体征。所有治疗方式均在不同时期导致肿瘤缩小和稳定，但均未完全根除肿瘤。18例患者中有14例（78%）在6个月至17年期间肿瘤大小持续缩小。5年总生存率和无进展生存率分别为80.0%和63.3%。56%的患者有内分泌后遗症，生长激素缺乏最为常见。2例患者死亡，均无神经纤维瘤病1型，患有下丘脑/视交叉肿瘤伴鞍上扩展及伴随的电解质异常。由于这些肿瘤进展缓慢且与内分泌病相关，我们建议如果疾病进展，化疗作为OPHG的主要治疗方法。
