关键词: cowden syndrome hamartomatous malformation lhermitte-duclos lhermitte-duclos disease pediatric neurosurgery

来  源:   DOI:10.7759/cureus.62076   PDF(Pubmed)

Abstract:
Lhermitte-Duclos disease (LDD), also known as dysplastic cerebellar gangliocytoma, is a rare, slow-growing, benign lesion that occurs in the cerebellum and is very uncommon in the pediatric population. There is a lack of literature and evidence about LDD management, and only one systematic review is available. Thus, more case reports and studies are warranted. This study reports a pediatric case diagnosed with LDD and describes the patient\'s clinical presentation, radiological findings, and histopathological criteria. In addition, important aspects of the disease are discussed to help reach the best management options. The main management option is surgical resection, though a \"wait and see\" approach is also an alternative, especially for asymptomatic patients. More studies are still needed to determine the best management options.
摘要:
Lhermitte-Duclos病(LDD),也被称为发育不良的小脑神经节细胞瘤,是一种罕见的,生长缓慢,发生在小脑的良性病变,在儿科人群中非常罕见。缺乏关于LDD管理的文献和证据,只有一份系统审查。因此,需要更多的病例报告和研究.本研究报告了诊断为LDD的儿科病例,并描述了患者的临床表现,放射学发现,和组织病理学标准。此外,讨论了该疾病的重要方面,以帮助达到最佳的管理选择。主要的管理选择是手术切除,尽管“观望”方法也是一种替代方法,尤其是无症状患者。仍需要更多的研究来确定最佳的管理方案。
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