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胚胎发育不良性神经上皮肿瘤:一种大脑皮质的良性但复杂的肿瘤。

Dysembryoplastic Neuroepithelial Tumor: A Benign but Complex Tumor of the Cerebral Cortex.

作者信息

Phi Ji Hoon, Kim Se Hoon

机构信息

Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

出版信息

Brain Tumor Res Treat. 2022 Jul;10(3):144-150. doi: 10.14791/btrt.2022.0015.

Abstract

Dysembryoplastic neuroepithelial tumor (DNET) is a distinct type of low-grade glioneuronal tumor. Clinically, DNET is highly associated with intractable epilepsy in young children and adolescents. Therefore, the burden of the tumor comprises oncological concerns (recurrence), seizure control, and quality of life. The pathology of DNET is characterized by glioneuronal elements and floating neurons. Grossly, many DNETs harbor separate nodules on the medial side of the mass. Some of the satellite lesions are tumor nodules that grow during the follow-up. Therefore, removing all satellite lesions may be important to prevent tumor progression. Seizure control is highly dependent on the complete removal of tumors, and the presence of satellite lesions also exerts a negative impact on seizure outcomes.

摘要

胚胎发育不良性神经上皮肿瘤(DNET)是一种独特类型的低级别神经胶质神经元肿瘤。临床上,DNET与幼儿及青少年的难治性癫痫高度相关。因此,该肿瘤的负担包括肿瘤学方面的问题(复发)、癫痫控制及生活质量。DNET的病理学特征为神经胶质神经元成分和漂浮神经元。大体上,许多DNET在肿块内侧有独立结节。一些卫星病灶是在随访期间生长的肿瘤结节。因此,切除所有卫星病灶对于预防肿瘤进展可能很重要。癫痫控制高度依赖于肿瘤的完全切除,卫星病灶的存在也会对癫痫治疗结果产生负面影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5049/9353162/7c99e41d5f46/btrt-10-144-g001.jpg

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