Department of Neurosurgery, Monash Health, Melbourne, Victoria, Australia; Department of Surgery, Monash University, Melbourne, Victoria, Australia.
Department of Surgery, Northern Hospital, Melbourne, Victoria, Australia.
World Neurosurg. 2022 Aug;164:424-435.e2. doi: 10.1016/j.wneu.2022.05.038. Epub 2022 May 14.
Craniopharyngiomas are morbid tumors that significantly reduce patients' quality of life (QoL). The lifelong burden of endocrine, visual, hypothalamic, and limbic dysfunction can have disastrous consequences for the physical and psychosocial health of patients. Elucidating the factors that influence QoL could guide therapeutic interventions to improve patient well-being.
A systematic review was performed in accordance with the PRISMA (preferred reporting items for systematic reviews and meta-analyses) statement using the PubMed and Medline databases. Studies that had reported patient QoL using validated metrics in both adult and pediatric populations were included. Bias and methodological rigor were assessed using the MINORS (methodological index for nonrandomized studies) criteria.
A total of 25 studies, including 2025 patients, were available for review. Most studies were small, retrospective, cohort studies with a high risk of bias. The QoL of the patients with craniopharyngioma was lower than that of the general population. Hypothalamic involvement was consistently the strongest predictor of QoL. Endocrinopathy contributed to morbidity but could be ameliorated by hormone replacement therapy. Social and emotional dysregulation and a poor memory are common complaints after surgery, and iatrogenic damage to the infundibulum, hypothalamus, limbic system, and frontal lobes might underlie these concerns. Sleep-wake cycle dysfunction and hypothalamic obesity are serious consequences of hypothalamic damage.
An experienced multidisciplinary team is necessary to optimally manage the complex cases of these patients. The poor QoL of patients with craniopharyngioma is multifactorial. However, the contribution of iatrogenesis is not insubstantial. Improved surgical techniques, focusing on hypothalamic preservation, and adjuvant treatment options are required to improve the well-being of these patients.
颅咽管瘤是一种严重影响患者生活质量（QoL）的恶性肿瘤。内分泌、视觉、下丘脑和边缘系统功能障碍的终身负担可能对患者的身心健康造成灾难性后果。阐明影响 QoL 的因素可以指导治疗干预措施，以改善患者的幸福感。
根据 PRISMA（系统评价和荟萃分析的首选报告项目）声明，使用 PubMed 和 Medline 数据库进行了系统评价。纳入了使用成人和儿科人群中经过验证的指标报告患者 QoL 的研究。使用 MINORS（非随机研究方法学指数）标准评估偏倚和方法学严谨性。
共有 25 项研究，包括 2025 名患者，可供审查。大多数研究是小型的、回顾性的队列研究，存在较高的偏倚风险。颅咽管瘤患者的 QoL 低于一般人群。下丘脑受累始终是 QoL 的最强预测因素。内分泌疾病会导致发病，但激素替代疗法可以减轻其影响。手术后常见的社会和情绪失调以及记忆力差，而漏斗、下丘脑、边缘系统和额叶的医源性损伤可能是这些问题的基础。睡眠-觉醒周期功能障碍和下丘脑性肥胖是下丘脑损伤的严重后果。
需要有经验的多学科团队来优化管理这些患者的复杂病例。颅咽管瘤患者 QoL 较差是多因素的。然而，医源性因素的影响不容忽视。需要改进手术技术，重点保护下丘脑，并提供辅助治疗选择，以提高这些患者的幸福感。
