Wang Mengmeng, Zhou Peizhi, Zhang Shizhen, Liu Xueyou, Lv Liang, Wang Zeming, Ye Feng, Ju Yan, Jiang Shu
Department of Neurosurgery, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu, 610041, People's Republic of China.
Department of Neurosurgery, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Childs Nerv Syst. 2018 Mar;34(3):423-430. doi: 10.1007/s00381-017-3632-6. Epub 2017 Oct 24.
Pediatric germ cell tumors (GCTs) involving the basal ganglia and thalamus are relatively rare neoplasms which have not been extensively described. We here summarize the clinical and radiological features of a series of such tumors and discuss optimal treatment strategies based upon our experience.
A total of 15 pediatric patients with basal ganglionic and thalamic GCTs were treated between 2011 and 2016 at West China Hospital. Epidemiological characteristics, clinical features, imaging findings, and treatment strategies were reviewed retrospectively.
GCTs constituted 28% (15/53) of pediatric basal ganglionic and thalamic tumors in our institution between 2011 and 2016. There were 12 males and 3 females with mean age of 11.7 ± 2.8 years (range, 7-16 years). The most common initial manifestation was hemiparesis (n = 13, 86.7%), followed by headache (n = 5, 33.3%), vomiting (n = 3, 20.0%), cognitive disturbance (n = 2, 13.3%), and seizure (n = 1, 6.7%). No tumors were incidentally detected. The mean duration of the symptoms before diagnosis was 4.4 ± 3.9 months (range from 9 days to 13 months). The maximum diameters of the lesions ranged from 3.2 to 6.5 cm (mean 4.7 ± 1.1 cm). Cysts were seen in tumors in MRIs in 11 patients (73%), intratumoral hemorrhages in 3 (20%), calcification in 2 (13%), and there was obstructive hydrocephalus in 1 (7%). Of note, hemiatrophy was observed in 9 cases (60.0%). The mean follow-up for the 15 patients was 28 months (range, 9-54 months), and no patients were lost. During the follow-up period, all patients (9 cases) with germinomas responded well to radiotherapy, and no recurrence was observed. Among 4 patients with mixed nongerminomatous germ cell tumor, 2 suffered tumor recurrence after treatment. Neurological deficits improved or remained unchanged in 12 patients but 3 developed new dysfunction including significant cognitive disturbance and hemiparesis.
Pediatric GCTs in the basal ganglia and thalamus are not as rare as previously considered. Tumor markers should be tested routinely for tumors in these sites in young patients. Optimal treatment strategy based on accurate diagnosis and comprehensive clinical assessment should be recommended.
累及基底神经节和丘脑的小儿生殖细胞肿瘤（GCTs）是相对罕见的肿瘤，尚未得到广泛描述。我们在此总结一系列此类肿瘤的临床和放射学特征，并根据我们的经验讨论最佳治疗策略。
2011年至2016年期间，共有15例患有基底神经节和丘脑GCTs的小儿患者在华西医院接受治疗。回顾性分析其流行病学特征、临床特征、影像学表现和治疗策略。
2011年至2016年期间，GCTs占我院小儿基底神经节和丘脑肿瘤的28%（15/53）。其中男性12例，女性3例，平均年龄11.7±2.8岁（范围7 - 16岁）。最常见的初始表现为偏瘫（n = 13，86.7%），其次是头痛（n = 5，33.3%）、呕吐（n = 3，20.0%）、认知障碍（n = 2，13.3%）和癫痫发作（n = 1，6.7%）。无肿瘤为偶然发现。诊断前症状的平均持续时间为4.4±3.9个月（范围从9天到13个月）。病变的最大直径范围为3.2至6.5厘米（平均4.7±1.1厘米）。11例患者（73%）的MRI显示肿瘤内有囊肿，3例（20%）有瘤内出血，2例（13%）有钙化，1例（7%）有梗阻性脑积水。值得注意的是，9例（60.0%）观察到偏侧萎缩。15例患者的平均随访时间为28个月（范围9 - 54个月），无患者失访。在随访期间，所有生殖细胞瘤患者（9例）对放疗反应良好，未观察到复发。在4例混合性非生殖细胞性生殖细胞肿瘤患者中，2例治疗后肿瘤复发。12例患者神经功能缺损改善或保持不变，但3例出现新的功能障碍，包括明显的认知障碍和偏瘫。
基底神经节和丘脑的小儿GCTs并不像以前认为的那么罕见。对于年轻患者这些部位的肿瘤应常规检测肿瘤标志物。应推荐基于准确诊断和综合临床评估的最佳治疗策略。
