小脑发育异常性神经节细胞瘤（Lhermitte-Duclos病）的临床视角
Clinical Perspective on Dysplastic Gangliocytoma of the Cerebellum (Lhermitte-Duclos Disease).
作者信息
Ma Jun, Jia Guijun, Chen Siyuan, Jia Wang
机构信息
Department of Neurosurgery, Beijing Tiantan Hospital affiliated to Capital Medical University, Beijing, China; National Clinical Research Center for Neurological Diseases, Center of Brain Tumor, Beijing Institute for Brain Disorders and Beijing Key Laboratory of Brian Tumor, Beijing, China.
Department of Neurosurgery, Beijing Tiantan Hospital affiliated to Capital Medical University, Beijing, China; National Clinical Research Center for Neurological Diseases, Center of Brain Tumor, Beijing Institute for Brain Disorders and Beijing Key Laboratory of Brian Tumor, Beijing, China.
出版信息
World Neurosurg. 2019 Feb;122:16-23. doi: 10.1016/j.wneu.2018.10.085. Epub 2018 Oct 23.
BACKGROUND
Dysplastic gangliocytoma of the cerebellum, also called Lhermitte-Duclos disease (LDD), is known as a rare, benign brain tumor. Around 200 cases have been reported.
CASE DESCRIPTION
Here we introduced a newly diagnosed adult case with intratumoral hemorrhage. Then we reviewed 18 cases diagnosed in our institute, including 12 adults and 6 children.
CONCLUSIONS
Preoperative diagnosis based on magnetic resonance imaging is 50% in adults and 16.7% in pediatrics. Diagnostic tiger stripes are not always seen in pediatric cases. The prognosis of the disease is good if total resection can be achieved. Further examinations for PTEN mutation and other comorbidities are recommended.
背景
小脑发育异常性神经节细胞瘤，也称为勒米特-迪克洛病（LDD），是一种罕见的良性脑肿瘤。已报道约200例病例。
病例描述
在此，我们介绍了1例新诊断的伴有肿瘤内出血的成年病例。然后我们回顾了我院诊断的18例病例，其中包括12例成人和6例儿童。
结论
基于磁共振成像的术前诊断在成人中为50%，在儿科患者中为16.7%。儿科病例中并不总是能看到诊断性的虎纹征。如果能实现全切除，该疾病的预后良好。建议进一步检查PTEN突变及其他合并症。
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