Pediatric Neurosurgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Campus Virchow Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany.
Department of Pediatric Oncology, University Children's Hospital Tübingen, Tübingen, Germany.
Childs Nerv Syst. 2024 Oct;40(10):3065-3074. doi: 10.1007/s00381-024-06498-2. Epub 2024 Jun 25.
The extent of resection of pediatric low-grade glioma mostly improves progression-free survival. In chiasmatic hypothalamic glioma (CHG), complete resections are limited due to the relevantly high risk of associated neurological and endocrinological deficits. Still, surgery might have its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcomes.
Medical records of surgically treated pediatric CHG patients between 2004 and 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival.
A total of 63 patients (33 female, NF-1, n = 8) were included. Age at first diagnosis was 4.6 years (range 0.2-16.9) and cohort follow-up was 108 ± 72 months. Twenty patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range 0.16-16.9). Patients received a median of 2 tumor surgeries (range 1-5). Cyst drainage was accomplished in 15 patients, and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow-up, 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range 0-100). Four patients died. Hormone substitution was necessary in 30.2%, and visual acuity was impaired in 66% of patients.
Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, limited tumor-debulking, cyst fenestration, and hydrocephalus management in the framework of MDT decision-making. Team experience contributes to reducing possible deficits in this challenging cohort.
最大限度地切除小儿低度神经胶质瘤可改善无进展生存期。在视交叉下丘脑神经胶质瘤（CHG）中，由于相关的神经和内分泌缺陷风险较高，完全切除受到限制。尽管如此，手术可能在多学科团队（MDT）方法的框架内发挥作用。我们报告了来自两个中心的手术选择及其对长期结果影响的回顾性经验。
分析了 2004 年至 2022 年间接受手术治疗的小儿 CHG 患者的病历。检索了患者特征、手术干预、组织学和非手术治疗以及视力、内分泌功能和生存等预后指标。
共纳入 63 例患者（33 例女性，NF-1，n=8）。首次诊断时的年龄为 4.6 岁（范围 0.2-16.9），队列随访时间为 108±72 个月。20 例患者接受活检手术治疗，43 例患者接受肿瘤切除术，中位年龄为 6.5 岁（范围 0.16-16.9）。患者接受了中位数为 2 次肿瘤手术（范围 1-5）。15 例患者进行了囊肿引流，27 例患者进行了脑室-腹腔分流术。69.8%的患者接受了非手术治疗。随访结束时，74.6%的患者疾病稳定。队列的中位 Karnofsky 评分为 90（范围 0-100）。4 例患者死亡。30.2%的患者需要激素替代治疗，66%的患者视力受损。
小儿 CHG 是一种慢性病，由于总体生存率高且多次进展，因此需要多学科团队决策，进行手术治疗。手术治疗仍然是一种关键的治疗选择，可提供活检、有限的肿瘤切除术、囊肿开窗术和脑积水管理。团队经验有助于减少在这一具有挑战性的患者群体中可能出现的缺陷。
