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Dysplastic Cerebellar Gangliocytoma(Lhermitte-Duclos Disease) Kouichirou OKAMOTO 1 , Manabu NATSUMEDA 2 , Makoto OISHI 2 , Yukihiko FUJII 2 1Department of Translational Research, Brain Research Institute, Niigata University 2Department of Neurosurgery, Brain Research Institute, Niigata University Keyword: 虎縞様層構造 , 過誤腫 , PTEN遺伝子変異 , カウデン症候群 , tiger-striped striation , hamartoma , PTEN mutations , Cowden syndrome pp.395-399
Published Date 2021/3/10
DOI https://doi.org/10.11477/mf.1436204404
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 Dysplastic cerebellar gangliocytoma or Lhermitte-Duclos disease(LDD)is a rare benign cerebellar lesion composed of dysplastic ganglion cells that conform to the existing cortical architecture. In this disease, the enlarged ganglion cells are predominantly located within the internal granular layer, and they thicken the cerebellar folia. The architecture of the affected cerebellar hemisphere with the enlarged cerebellar folia and the cystic changes, in some cases, present as “tiger-striped striations,” a characteristic imaging finding that is not specific to LDD. This imaging feature may be observed in medulloblastoma and isolated cerebellar Rosai-Dorfman disease.

 This cerebellar lesion is a major central nervous system manifestation of Cowden syndrome, an autosomal dominant condition that causes various hamartomas and neoplasms. A molecular-based study estimated the prevalence of Cowden syndrome to be 1 case per 200,000. In a study involving 211 patients with Cowden syndrome, 32% developed LDD.

 LDD can be diagnosed in young children and older adults within the eighth decades of life. PTEN mutations have been identified in virtually all adult-onset LDDs, but not in childhood-onset cases.


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電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院

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