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与原始持续性三叉神经动脉相关的鞍内脊索瘤。

Intrasellar chordoma associated with a primitive persistent trigeminal artery.

作者信息

Navas Marta, Martinez Pedro, Shakur Sophia F, Barbosa Antonio, Barcena Eduardo, Gordillo Carlos, Fraga Javier, Blanco Concepcion, Sola Rafael G

机构信息

La Princesa University Hospital, Department of Neurosurgery, Madrid, Spain.

出版信息

Turk Neurosurg. 2015;25(1):146-53. doi: 10.5137/1019-5149.JTN.8759-13.1.

Abstract

Chordomas located primarily in the sellar region are uncommon, and may be misdiagnosed non-functioning pituitary adenoma. Furthermore, the association of a persistent primitive trigeminal artery (PPTA) with an intrasellar chordoma is extremely rare, and no similar cases have been reported in the literature to date. The coexistence of intrasellar chordoma (ISC) and PPTA makes safe and complete tumor resection challenging, and preoperative endovascular occlusion of this artery may be helpful. We report a case of a 32-year-old man who developed right hemifacial paresthesias and a cranial nerve six palsy. MRI study demonstrated the presence of a primary ISC associated with a PPTA. Angiographic balloon test occlusion of the PPTA revealed no neurological changes, so this vessel was endovascularly occluded by coiling. The lesion was subtotally removed through a sublabial transsphenoidal approach, without intraoperative bleeding complications. Histological examination of the lesion was consistent with the diagnosis of chordoma. Detailed preoperative neurovascular evaluation may be useful to detect vascular anomalies associated with intracranial chordomas, such as PPTA. In this report we emphasize the importance of appropriate treatment of vascular anomalies prior intrasellar lesions resection that may facilitate surgery and avoid potential hazardous intraoperative bleeding complications.

摘要

主要位于鞍区的脊索瘤并不常见,可能会被误诊为无功能垂体腺瘤。此外,持续性原始三叉动脉(PPTA)与鞍内脊索瘤相关联的情况极为罕见,迄今为止文献中尚未报道过类似病例。鞍内脊索瘤(ISC)与PPTA并存使得安全完整地切除肿瘤具有挑战性,术前对该动脉进行血管内闭塞可能会有所帮助。我们报告一例32岁男性,出现右侧面部感觉异常和展神经麻痹。MRI检查显示存在与PPTA相关的原发性ISC。对PPTA进行血管造影球囊试验闭塞未发现神经功能改变,因此通过线圈栓塞对该血管进行了血管内闭塞。通过唇下经蝶窦入路对病变进行了次全切除,术中无出血并发症。病变的组织学检查与脊索瘤诊断一致。详细的术前神经血管评估可能有助于检测与颅内脊索瘤相关的血管异常,如PPTA。在本报告中,我们强调在切除鞍内病变之前对血管异常进行适当治疗的重要性,这可能有助于手术并避免潜在的术中危险出血并发症。

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