小脑发育异常性神经节细胞瘤：后颅窝占位性病变中罕见的鉴别诊断。
Dysplastic gangliocytoma of the cerebellum: rare differential diagnosis in space occupying lesions of the posterior fossa.
作者信息
Buhl R, Barth H, Hugo H-H, Straube T, Mehdorn H M
机构信息
Department of Neurosurgery, University of Kiel, Germany.
出版信息
Acta Neurochir (Wien). 2003 Jun;145(6):509-12; discussion 512. doi: 10.1007/s00701-003-0040-3.
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos) is a rare space occupying lesion of the posterior fossa with some typical neuroradiological features which can be better diagnosed in the MRI era. This is the major CNS manifestation of Cowden disease. In recent years more publications underlined the association of these hamartomatous lesions. We add another patient with a dysplastic gangliocytoma of the cerebellum who also had a thyroid adenoma and fulfilled the criteria of having Cowden disease.A problem of surgical removal of these tumors is missing the borderline between tumor and healthy cerebellum tissue so that incomplete removal of the tumor is not rare. Surgical removal of these tumors in an open MRI unit would be a good indication.
小脑发育异常性神经节细胞瘤（Lhermitte-Duclos病）是一种罕见的后颅窝占位性病变，具有一些典型的神经放射学特征，在MRI时代能得到更好的诊断。这是考登病的主要中枢神经系统表现。近年来，更多的出版物强调了这些错构瘤性病变之间的关联。我们又增加了一名患有小脑发育异常性神经节细胞瘤的患者，该患者还患有甲状腺腺瘤，符合考登病的标准。手术切除这些肿瘤的一个问题是难以区分肿瘤与健康小脑组织的边界，因此肿瘤切除不完全的情况并不少见。在开放式MRI设备中对这些肿瘤进行手术切除将是一个很好的选择。
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