Dysplastic cerebellar gangliocytoma/Lhermitte-Duclos disease: report of 6 cases and review of the literatures

VernacularTitle:小脑发育不良性节细胞瘤/Lhermitte-Duclos病6例临床病理分析并文献复习
Author: Ju YUAN ¹ ; Nan WU ; Xuan WANG ; shan Shan SHI ; feng Zhen LU ; rong Jin WU
Author Information

1. 中国人民解放军南京总医院病理科
Keywords: dysplastic cerebellar gongliocytoma; Lhermitte-Duclos disease; Cowden syndrome; PTEN mutation
From: Chinese Journal of Clinical and Experimental Pathology 2017;33(9):1001-1004
CountryChina
Language:Chinese
Abstract: Purpose To investigate the clinicopthologic features of dysplastic cerebellar gongliocytoma/Lhermitte-Duclos disease (LDD) and to discuss the diagnosis and differential diagnosis of the tumor.Methods Histopathological characteristics and immunohistochemical of SP results of 6 cases of LDD were studied and the relevant literatures were reviewed.Results 6 cases ranged in age from 23 to 56 years old,with an average of 34 years.The clinical manifestations were intracranial hypertension with or without cerebellar signs.MRI manifestations were characteristic by "tiger spots".Histology showed local cerebellar structure disorders,granulocytes and Purkinje cells decreased and replaced by a large number of abnormal ganglion cells.2 cases were followed up for 5 to 8 years recurrence,the remaining 4 cases recovered well.Conclusion LDD is a rare primary benign lesions of the cerebellum.Diagnosis depends on imaging and histopathological examination.The gross total resection is the best treatment choice.