关键词: CMS cerebellar mutism syndrome growth pattern medulloblastoma neurological sequelae oncology pediatric neurosurgery resection

来  源:   DOI:10.3171/2019.11.PEDS19349

Abstract:
Extensive resection of a tumor in the posterior fossa in children is associated with the risk of neurological deficits. The objective of this study was to prospectively evaluate the short-term neurological morbidity in children after medulloblastoma surgery and relate this to the tumor\'s growth pattern and to the extent of resection.
In 160 patients taking part in the HIT-SIOP PNET 4 (Hyperfractionated Versus Conventionally Fractionated Radiotherapy in Standard Risk Medulloblastoma) trial, neurosurgeons prospectively responded to questions concerning the growth pattern of the tumor they had resected. The extent of resection (gross, near, or subtotal) was evaluated using MRI. The patients\' neurological status before resection and around 30 days after resection was recorded.
Invasive tumor growth, defined as local invasion in the brain or meninges, cranial nerve, or major vessel, was reported in 58% of the patients. After surgery almost 70% of all patients were affected by one or several neurological impairments (e.g., impaired vision, impaired extraocular movements, and ataxia). However, this figure was very similar to the preoperative findings. Invasive tumor growth implied a significantly higher number of impairments after surgery (p = 0.03) and greater deterioration regarding extraocular movements (p = 0.012), facial weakness (p = 0.048), and ataxia in the arms (p = 0.014) and trunk (p = 0.025) compared with noninvasive tumor growth. This deterioration was not dependent on the extent of resection performed. Progression-free survival (PFS) at 5 years was 80% ± 4% and 76% ± 5% for patients with invasive and noninvasive tumor growth, respectively, with no difference in the 5-year PFS for extent of resection.
Preoperative neurological impairments and invasive tumor growth were strong predictors of deterioration in short-term neurological outcome after medulloblastoma neurosurgery, whereas the extent of resection was not. Neither tumor invasiveness nor extent of resection influenced PFS. These findings support the continuation of maximal safe resection in medulloblastoma surgery where functional risks are not taken in areas with tumor invasion.
摘要:
目的:儿童后颅窝肿瘤的广泛切除与神经功能缺损的风险相关。这项研究的目的是前瞻性评估儿童髓母细胞瘤手术后的短期神经系统发病率,并将其与肿瘤的生长模式和切除程度联系起来。
方法:在参加HIT-SIOPPNET4(标准风险髓母细胞瘤的超分割与常规分割放射治疗)试验的160名患者中,神经外科医生前瞻性地回答了有关他们切除的肿瘤生长模式的问题.切除的程度(总体,近,或小计)使用MRI进行评估。记录患者切除前和切除后约30天的神经状况。
结果:侵袭性肿瘤生长,定义为大脑或脑膜的局部侵入,颅神经,或者主要船只,在58%的患者中报告。手术后,几乎70%的所有患者都受到一种或多种神经系统损害的影响(例如,视力受损,眼外运动受损,和共济失调)。然而,这个数字与术前发现非常相似.侵袭性肿瘤生长意味着手术后损伤的数量显著增加(p=0.03)和眼外运动的恶化更大(p=0.012),面部无力(p=0.048),与非侵入性肿瘤生长相比,手臂(p=0.014)和躯干(p=0.025)的共济失调。这种恶化不取决于所进行的切除程度。侵袭性和非侵袭性肿瘤生长患者的5年无进展生存率(PFS)分别为80%±4%和76%±5%。分别,切除程度的5年PFS没有差异。
结论:术前神经损伤和浸润性肿瘤生长是髓母细胞瘤神经外科术后短期神经预后恶化的强预测因子,而切除的程度没有。肿瘤侵袭性和切除程度均不影响PFS。这些发现支持在髓母细胞瘤手术中继续进行最大的安全切除,在肿瘤浸润区域不存在功能风险。
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