不寻常的小脑肿块的不寻常表现:发育不良的小脑神经节细胞瘤/ hermitte duclos病——文献复习并报告2例

P. Sinha, R. H. Reddy, Priyanka.
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摘要

Lhermitte-Duclos病或小脑发育不良神经节细胞瘤是一种罕见的小脑皮质单侧良性肿块,具有独特的MRI诊断特征,典型的“虎纹”外观。鉴于可能与考登综合征相关,因此需要识别并发恶性肿瘤,对疾病的正确认识尤为重要。MRI是一种公认的诊断方法,由于其独特的成像特征,有助于区分LDD。报告了两例活检证实的这种不寻常疾病的不典型表现。不典型表现包括一例发育不良的左小脑半球弥散受限,另一例累及双侧小脑半球和小脑蚓部。
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Unusual presentation of an unusual cerebellar mass: dysplastic cerebellar gangliocytoma/lhermitte duclos disease-review of literature and report of 2 cases
Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is a rare benign unilateral mass of the cerebellar cortex having unique diagnostic MRI features characterized by classic ‘tiger striped’ appearance. Correct recognition of the disease is particularly important given the possible correlation with cowden syndrome and therefore the need to identify concurrent malignancies. MRI is a considered diagnostic and is helpful in distinguishing LDD due to its unique imaging features. Two biopsy proven cases of this unusual disease were reported with atypical presentations. Atypical presentations includepresence of diffusion restriction in the dysplastic left cerebellar hemisphere in one case and involvement of bilateral cerebellar hemisphere and vermis in the second one.
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