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儿童弥漫性软脑膜神经胶质神经元肿瘤。

Diffuse Leptomeningeal Glioneuronal Tumor of Childhood.

机构信息

From the Department of Radiology (D.A.L., P.F.), School of Medicine, West Virginia University, Morgantown, West Virginia.

Department of Neuroradiology (D.A.L., P.F.), Rockefeller Neuroscience Institute, WV University, Morgantown, West Virginia.

出版信息

AJNR Am J Neuroradiol. 2020 Nov;41(11):2155-2159. doi: 10.3174/ajnr.A6737. Epub 2020 Sep 10.

Abstract

Diffuse leptomeningeal glioneuronal tumor is a newly defined entity under the neuronal and mixed neuronal-glial tumors category in the 2016 World Health Organization classification of brain tumors. In this series, we report clinical, radiologic, and histologic findings in 7 cases of diffuse leptomeningeal glioneuronal tumor. Our cases and literature review indicate that the most characteristic imaging finding is diffuse intracranial and intraspinal nodular leptomeningeal thickening and enhancement. This is often associated with small cyst-like, nonenhancing lesions. It should be noted that tumors sometimes bear nontypical features, for example, presenting as a solitary spinal cord mass without leptomeningeal involvement or with a dominant intracranial mass. In children with characteristic imaging findings and without clinical features of infection, the radiologist has an opportunity to promptly raise the possibility of diffuse leptomeningeal glioneuronal tumor, and thereby, affect streamlined diagnostic evaluation.

摘要

弥漫性软脑膜神经胶质神经元肿瘤是 2016 年世界卫生组织脑肿瘤分类中新定义的神经元和混合性神经元-神经胶质肿瘤类别中的一个实体。在本系列中,我们报告了 7 例弥漫性软脑膜神经胶质神经元肿瘤的临床、影像学和组织学发现。我们的病例和文献复习表明,最具特征性的影像学发现是弥漫性颅内和脊髓结节性软脑膜增厚和强化。这通常与小的囊状、不增强病变有关。需要注意的是,肿瘤有时具有非典型特征,例如表现为无软脑膜受累的孤立性脊髓肿块,或伴有颅内占位性病变为主。对于具有特征性影像学表现且无感染临床特征的儿童,放射科医生有机会及时提出弥漫性软脑膜神经胶质神经元肿瘤的可能性,从而影响简化的诊断评估。

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