Clarke Michelle J, Foy Andrew B, Wetjen Nicholas, Raffel Corey
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Neurosurg Focus. 2006 Jan 15;20(1):E5. doi: 10.3171/foc.2006.20.1.6.
Subependymal giant cell astrocytomas (SEGAs) are a common manifestation of tuberous sclerosis (TS). These evolving tumors have a propensity to cause obstructive hydrocephalus, usually due to obstruction at the level of the foramen of Monro. Differentiating SEGAs from subependymal nodules (SENs) before obstruction occurs may improve the morbidity associated with these tumors. In this study the authors' aim was to determine imaging characteristics of proven tumors in a single-center pediatric population.
The authors retrospectively reviewed all records and images obtained in patients with TS in whom results of biopsy sampling had proven that their tumors were SEGAs. Time to presentation, signs and symptoms at presentation, and imaging characteristics of the evolving tumors were noted. Twelve patients with 14 SEGAs proven by the results of biopsy sampling were reviewed. Resection was recommended for symptomatic and neuroimaging evidence of hydrocephalus (41%), tumor growth without evidence of hydrocephalus (33%), and for poorly controlled seizures (25%). The mean diameter of the tumors at the time of resection was 1.9 cm (range 0.3-4 cm), and no tumor recurred. Because of the pathological and radiographic continuum of SENs and SEGAs, it remains difficult to predict whether and when a given lesion will progress. Tumor growth and contrast enhancement are the most common signs of progression on neuroimages, and may be seen prior to the development of obstructive hydrocephalus.
Patients with SENs and SEGAs should undergo follow-up neuroimaging at yearly intervals, and if lesions show signs of progression (contrast enhancement or growth), these intervals should be shortened and consideration given to early resection.
室管膜下巨细胞星形细胞瘤(SEGA)是结节性硬化症(TS)的常见表现。这些不断发展的肿瘤容易导致梗阻性脑积水,通常是由于室间孔水平的梗阻所致。在梗阻发生前将SEGA与室管膜下结节(SEN)区分开来,可能会改善与这些肿瘤相关的发病率。在本研究中,作者的目的是确定单中心儿科人群中已确诊肿瘤的影像学特征。
作者回顾性分析了所有结节性硬化症患者的记录和影像资料,其活检样本结果证实肿瘤为SEGA。记录了就诊时间、就诊时的体征和症状以及不断发展的肿瘤的影像学特征。对12例经活检样本结果证实患有14个SEGA的患者进行了回顾。对于有症状且有脑积水神经影像学证据的患者(41%)、无脑积水证据的肿瘤生长患者(33%)以及癫痫控制不佳的患者(25%),建议进行手术切除。切除时肿瘤的平均直径为1.9厘米(范围0.3 - 4厘米),且无肿瘤复发。由于SEN和SEGA在病理和影像学上具有连续性,仍然难以预测给定病变是否以及何时会进展。肿瘤生长和对比增强是神经影像上最常见的进展迹象,可能在梗阻性脑积水发生之前出现。
患有SEN和SEGA的患者应每年接受一次随访神经影像学检查,如果病变出现进展迹象(对比增强或生长),应缩短检查间隔并考虑早期切除。