Department of Neurosurgery, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
Department of Neurosurgery and Neurooncology, First Faculty of Medicine, Charles University and Military University Hospital, Prague, Czech Republic.
Acta Neurochir (Wien). 2023 Dec;165(12):4279-4292. doi: 10.1007/s00701-023-05729-w. Epub 2023 Aug 3.
Tumors of the fourth ventricle are frequently treated pathologies in pediatric neurosurgery. Data regarding predictors for permanent neurological deficits, long-term functional outcomes, cerebellar mutism (CM), the extent of resection (EOR), and oncological outcomes are scarce. We attempt to contribute to this topic with an analysis of our institutional cohort.
A retrospective single-center study of patients aged ≤ 19 years who underwent primary surgical resection of a fourth ventricular tumor over a 15-year period (2006-2021). Predictors analyzed included age, gender, surgical approach, anatomical pattern, tumor grade, EOR, tumor volume, and others as appropriate.
One hundred six patients were included (64 males, mean age 7.3 years). The rate of permanent neurological deficit was 24.2%; lateral tumor extension (p = 0.036) and tumor volume greater than 38 cm (p = 0.020) were significant predictors. The presence of a deficit was the only significant predictor of reduced (less than 90) Lansky score (p = 0.005). CM occurred in 20.8% of patients and was influenced by medulloblastoma histology (p = 0.011), lateral tumor extension (p = 0.017), and male gender (p = 0.021). No significant difference between the transvermian and telovelar approach in the development of CM was detected (p = 0.478). No significant predictor was found for the EOR. EOR was not found to be a significant predictor of overall survival for both low-grade and high-grade tumors; however, gross total resection (GTR) was protective against tumor recurrence compared to near-total or subtotal resection (p < 0.001). In addition, survival was found to be better in older patients (≥ 7.0 years, p = 0.019).
The overall rate of postoperative complications remains high due to the eloquent localization. Older patients (> 7 years) have been found to have better outcomes and prognosis. Achieving GTR whenever feasible and safe has been shown to be critical for tumor recurrence. CM was more common in patients with medulloblastoma and in patients with tumors extending through the foramen of Luschka. The telovelar approach uses a safe and anatomically sparing corridor; however, it has not been associated with a lower incidence of CM and neurological sequelae in our series, showing that each case should be assessed on an individual basis.
第四脑室肿瘤是小儿神经外科常见的治疗性病变。关于永久性神经功能缺损、长期功能结果、小脑缄默症（CM）、切除程度（EOR）和肿瘤学结果的预测因素的数据很少。我们试图通过对我们机构队列的分析来对此主题做出贡献。
这是一项回顾性单中心研究，纳入了在 15 年内（2006-2021 年）接受原发性第四脑室肿瘤手术切除的年龄≤19 岁的患者。分析的预测因素包括年龄、性别、手术入路、解剖模式、肿瘤分级、EOR、肿瘤体积等。
共纳入 106 例患者（64 例男性，平均年龄 7.3 岁）。永久性神经功能缺损的发生率为 24.2%；外侧肿瘤延伸（p=0.036）和肿瘤体积大于 38cm（p=0.020）是显著的预测因素。存在缺陷是降低（小于 90）兰斯基评分的唯一显著预测因素（p=0.005）。20.8%的患者出现 CM，受髓母细胞瘤组织学（p=0.011）、外侧肿瘤延伸（p=0.017）和男性性别（p=0.021）的影响。在 CM 的发展中，横纤维和远隔经小脑蚓部入路之间没有发现显著差异（p=0.478）。EOR 没有发现是低级别和高级别肿瘤总生存率的显著预测因素；然而，与近全切除或次全切除相比，大体全切除（GTR）对肿瘤复发具有保护作用（p<0.001）。此外，发现年龄较大（≥7.0 岁，p=0.019）的患者生存情况更好。
由于位置表浅，术后并发症的总体发生率仍然很高。研究发现年龄较大（>7 岁）的患者具有更好的结局和预后。只要可行且安全，实现 GTR 一直被认为对肿瘤复发至关重要。在我们的研究中，CM 在髓母细胞瘤患者和肿瘤延伸穿过 Luschka 孔的患者中更为常见。远隔经小脑蚓部入路使用安全且解剖保留的通道；然而，在我们的系列中，它与 CM 和神经后遗症的发生率较低无关，表明每个病例都应根据具体情况进行评估。
