目的:小儿低度胶质瘤的切除程度主要提高了无进展生存期。在交叉下丘脑胶质瘤(CHG)中,由于相关的神经和内分泌缺陷的相关高风险,完全切除是有限的。尽管如此,手术可能在多学科团队(MDT)方法的框架中发挥作用.我们报告了两个中心关于手术选择及其对长期结局的影响的回顾性经验。
方法:分析了2004年至2022年间手术治疗的小儿CHG患者的病历。患者特征,手术干预,组织学,和非手术治疗一起检索结果测量,如视力,内分泌功能,和生存。
结果:共有63名患者(33名女性,包括NF-1,n=8)。首次诊断的年龄为4.6岁(范围0.2-16.9),队列随访为108±72个月。20例患者接受了活检手术治疗,43例患者的中位年龄为6.5岁(范围为0.16-16.9)。患者接受2次肿瘤手术的中位数(范围1-5)。15例患者完成囊肿引流,27例患者进行了脑室-腹腔分流术植入。非手术治疗占69.8%。在后续行动结束时,74.6%的患者病情稳定。该队列的中位Karnofsky评分为90(范围0-100)。四名患者死亡。30.2%需要激素取代,66%的患者视力受损。
结论:小儿CHG是一种慢性疾病,因为总体生存率高,有多个进展。手术治疗仍然是提供活检的关键治疗选择,有限的肿瘤减瘤,囊肿开窗术,以及MDT决策框架内的脑积水管理。团队经验有助于减少这个具有挑战性的队列中可能的赤字。
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.