Krisht Khaled M, Palmer Cheryl A, Osborn Anne G, Couldwell William T
Department of Neurosurgery, Clinical Neurosciences Center; and.
J Neurosurg Pediatr. 2013 Oct;12(4):328-33. doi: 10.3171/2013.5.PEDS1395. Epub 2013 Aug 2.
The authors describe a rare case of giant ecchordosis physaliphora (EP) in a 16-year-old female patient who presented with diplopia. Magnetic resonance imaging of the brain with Gd contrast revealed a 3.0 × 1.7 × 1.8-cm nonenhancing, extraaxial epidural mass along the dorsal aspect of the clivus that was T2 hyperintense and T1 isointense with intermediate signal on diffusion sequences. Resection via a transnasal transsphenoidal approach to the ventral clival wall resulted in a stable tumor size with no evidence of interval growth after 30 months. Although this case features a strictly extradural EP, this tumor more commonly occurs in the subdural space and requires differentiation from intradural chordoma. Unlike EP, intradural chordoma may enhance with Gd contrast, is more likely to be associated with cranial nerve palsies and brainstem symptoms, and will occasionally have an elevated MIB-1 index. In this paper the authors highlight the different possible midline locations for both EP and chordoma, the difficulty in distinguishing between intradural giant EP and intradural chordoma, and the potential occurrence of these lesions in young people despite their typically slow rate of growth, while also underscoring the need for further investigation into the tumors' cytogenetic behavior.
作者描述了一例罕见的巨大泡状脊索瘤（EP），患者为一名16岁女性，表现为复视。脑部磁共振成像增强扫描显示，在斜坡背侧有一个3.0×1.7×1.8厘米的无强化轴外硬膜外肿块，T2加权像呈高信号，T1加权像呈等信号，扩散序列呈中等信号。经鼻经蝶窦入路至斜坡腹侧壁进行切除，术后30个月肿瘤大小稳定，无间隔生长迹象。尽管该病例为严格的硬膜外EP，但这种肿瘤更常见于硬膜下间隙，需要与硬膜内脊索瘤相鉴别。与EP不同，硬膜内脊索瘤增强扫描可能有强化，更易伴有颅神经麻痹和脑干症状，且偶尔MIB-1指数会升高。在本文中，作者强调了EP和脊索瘤在中线的不同可能位置、区分硬膜内巨大EP和硬膜内脊索瘤的困难，以及尽管这些病变通常生长缓慢，但仍可能发生在年轻人中，同时还强调了对肿瘤细胞遗传学行为进行进一步研究的必要性。
