5-氨基酮戊酸辅助下小儿胚胎发育不良性神经上皮肿瘤切除术：病例说明
5-Aminolevulinic acid-assisted resection of pediatric dysembryoplastic neuroepithelial tumor: illustrative case.
作者信息
Beauchamp Luke H, Bercu Marian Michael, Avellino Anthony M
机构信息
College of Human Medicine, Michigan State University, East Lansing, Michigan; and.
Pediatric Neurosurgery, Helen DeVos Children's Hospital, Spectrum Health, Grand Rapids, Michigan.
出版信息
J Neurosurg Case Lessons. 2021 Jul 26;2(4):CASE20153. doi: 10.3171/CASE20153.
BACKGROUND
5-Aminolevulinic acid (5-ALA) is approved as an adjunct for the resection of high-grade gliomas and is associated with improved outcomes. Dysembryoplastic neuroepithelial tumors (DNETs) are benign glioneural tumors occurring primarily in pediatric patients and often manifesting with seizure disorder. The goal of the surgical intervention is to obtain gross-total resection, which is associated, in the majority of cases, with seizure freedom.
OBSERVATIONS
The authors present the first case report of a pediatric patient who underwent gross-total resection of a 5-ALA-positive DNET with no evidence of recurrent seizures (Engel class I).
LESSONS
Fluorescence-guided surgery using 5-ALA facilitated gross-total resection of the mass.
背景
5-氨基乙酰丙酸（5-ALA）被批准作为高级别胶质瘤切除的辅助药物，且与改善预后相关。胚胎发育不良性神经上皮肿瘤（DNETs）是主要发生于儿童患者的良性神经胶质肿瘤，常表现为癫痫发作。手术干预的目标是实现大体全切，在大多数情况下，这与癫痫发作缓解相关。
观察结果
作者报告了首例接受5-ALA阳性DNET大体全切且无癫痫复发证据（Engel I级）的儿科患者。
经验教训
使用5-ALA的荧光引导手术有助于肿瘤的大体全切。
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