Department of Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital, Via Mario Fiore n.6, 80129 Naples, Italy.
J Neurooncol. 2011 May;102(3):499-508. doi: 10.1007/s11060-010-0341-3. Epub 2010 Aug 20.
Gangliogliomas originating in the optic pathway are rare, with less than 20 cases reported in the literature. Diffuse, bilateral involvement of the entire optico-chiasmatic pathway is exceptional. We report a case of suprasellar ganglioglioma that involved bilaterally the entire pregeniculate optic pathway. The patient presented with visual deficit, nystagmus, papilledema and acute biventricular hydrocephalus secondary to intraventricular cyst that required urgent surgery. Endoscopic fenestration of the tumoral cyst allowed control of hydrocephalus and decompression of the visual pathway. Through microsurgical procedure by pterional approach, partial removal of the tumor and histological diagnosis were accomplished 1 week later. The patient was managed with chemotherapy and radiation therapy. He presents stable residual disease at 4-year follow-up. Embryological origins, histological features, neuroradiological appearance, management and prognosis of optic pathway gangliogliomas are reviewed.
起源于视神经通路的神经节细胞瘤很少见，文献中报道的病例少于 20 例。弥漫性、双侧累及整个视交叉通路的情况非常罕见。我们报告了一例鞍上神经节细胞瘤的病例，该肿瘤双侧累及整个视节前段视神经通路。患者表现为视力减退、眼球震颤、视盘水肿和急性双侧脑室积水，继发于脑室囊肿，需要紧急手术。通过内镜开窗术处理肿瘤性囊肿，可控制脑积水并对视路进行减压。1 周后，通过翼点入路的显微外科手术，部分切除了肿瘤并进行了组织学诊断。患者接受了化疗和放疗。4 年随访时，患者病情稳定，有残留病变。我们回顾了视神经通路神经节细胞瘤的胚胎起源、组织学特征、神经影像学表现、治疗和预后。
