Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland.
Folia Neuropathol. 2012;50(4):407-12. doi: 10.5114/fn.2012.32375.
Chordomas are rare tumours arising from notochordal remnants. Classical chordomas are generally extradural and, despite benign histopathology, they typically destroy the clivus and surrounding bone structures. Intradural lesions are extremely rare and less than thirty cases of intracranial, exclusively intradural chordomas have been reported so far. The intracranial, intradural but extranotochordal location of chordoma is extremely unique. The authors present a case of chordoma in intracranial location that clinically mimics lateral sphenoid wing meningioma. A previously healthy 39-year-old man was admitted to our Department because of optic disc oedema without neurological deficits. Neuroimaging studies showed a large, contrast-enhanced tumour in the right frontotemporal region that was thought to be a pterional meningioma. The patient underwent successful removal of the tumour. Histopathological study revealed a typical pattern of chordoma, confirmed by immunohistochemical findings. Because of the tumour location the differentiation between chordoma and chordoid meningioma ought to be considered. Such cases, including the present one, may lead to the conclusion that embryonic notochordal remnants may be lost in different places, even away from the neuroaxis.
脊索瘤是一种罕见的源自脊索残余组织的肿瘤。经典脊索瘤通常为硬膜外肿瘤，尽管组织学上为良性，但它们通常会破坏斜坡和周围的骨结构。硬膜内病变极为罕见，迄今为止，仅报告了不到 30 例颅内、完全硬膜内脊索瘤。脊索瘤位于颅内且位于硬膜外但不在脊索内的位置极为独特。作者报告了一例颅内脊索瘤病例，该病例在临床上类似于蝶骨翼外侧脑膜瘤。一名 39 岁健康男性因视神经盘水肿但无神经功能缺损而被收入我科。神经影像学研究显示右侧额颞区有一个大的、增强对比的肿瘤，考虑为翼点开颅脑膜瘤。患者成功地切除了肿瘤。组织病理学研究显示出典型的脊索瘤模式，免疫组织化学结果也证实了这一点。由于肿瘤的位置，需要考虑脊索瘤和脊索样脑膜瘤之间的鉴别。包括本例在内的此类病例可能得出这样的结论：胚胎脊索残余组织可能会在不同的位置丢失，甚至远离中枢神经系统。
