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病例学习 | 椎管-神经鞘瘤

时间:2022-07-15 00:14:56

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病例学习 | 椎管-神经鞘瘤

CASE 1

【临床病史】:患者,61岁男性出现进行性的下肢无力。61-year-old male with progressive lower extremity weakness

【影像图片】MRI图像

【影像表现】:

61-year-old male with a multilevel intradural extramedullary lesion demonstrating isointensity on sagittal T1 (Figure 1), intense enhancement on sagittal T1 post gad (Figure 2) and sagittal T1 post gad fat sat (Figure 3), and high signal intensity on sag STIR (Figure 4) (red arrows). 61岁男性伴有连续多个椎体平面的髓外硬膜下病变,T1`序列呈等信号(图1),T1增强扫描(图2)和T1压脂增强扫描(图3)显示病变明显强化,STir序列显示病变呈高信号(图4)

Axial T1 pre contrast (Figure 5) post contrast (Figure 6) and post contrast with fat saturation (Figure 7) demonstrate an intradural extramedullary lesion compressing and displacing the thoracic cord to the left (yellow arrows). 轴位T1平扫(图5)和增强扫描(图6)以及T1压脂增强扫描(图7)显示一个髓外硬膜下肿块向左侧推移挤压胸髓

【影像诊断】:Spinal Schwannoma 椎管神经鞘瘤

【诊断要点】:The differential diagnosis for intradural-extramedullary spinal mass includes nerve sheath tumor, meningioma, metastasis, paraganglioma, vascular malformations, inflammatory process, and developmental lesions. 脊椎髓外硬膜下肿块的鉴别诊断包括神经鞘瘤、脊膜瘤、转移瘤、副神经节瘤、血管畸形、炎性病变以及发育性病变。

Nerve sheath tumors and meningiomas demonstrate characteristics that may assist one in forming a differential diagnosis hierarchy on the basis of craniocaudal location, hyperintensity and heterogeneity of T2W images, intensity and heterogeneity of enhancement, and the presence of the “dural tail sign.” 神经鞘瘤和脊膜瘤显示的特征性表现可以帮助我们建立一个鉴别诊断体系,这居于病变的部位、T2序列上信号的强度和均匀性、强化程度和均匀性以及是否有脑膜尾征。

A spinal intradural-extramedullary mass that demonstrates heterogeneity, hyperintensity on T2W images or intense enhancement without the “dural tail sign” should be considered as a schwannoma; otherwise the diagnosis of meningioma is more probable.假如一个脊椎髓外硬膜下肿块在T2序列上表现为不均匀的高信号或有明显的强化而不伴有脑膜尾征时,应该首先考虑神经鞘瘤,反之需要考虑脑膜瘤。

【讨论】:nerve sheath tumors compromise schwannomas and neurofibromas, most often present in the fourth and fifth decades of life, and may be associated with neurofibromatosis. Schwannomas arise from dorsal sensory roots, more commonly in the lower thoracic and lumbar areas. Neurofibromas, unlike schwannomas, involve the parent nerve, and are unencapsulated.

神经鞘瘤又可以分为许旺氏细胞瘤和神经纤维瘤,大部分见于40-50岁,可能伴有神经纤维瘤病。许旺氏细胞瘤起源于背侧神经根,更常见于下段胸椎和腰椎区域,而神经纤维瘤常常累及神经根,且是无包膜的。

Meningiomas are the second most common intradural-extramedullary spinal tumor and are more common in women. Multiple meningiomas may also be associated with neurofibromatosis. Meningiomas are predominately located in the upper and mid- thoracic areas, different from schwannomas, but are in a similar distribution in the anterior-posterior plane. Both nerve sheath tumors and meningiomas can be found in a “dumbbell shape,” with both extra- and intradural components.

脊膜瘤是第二常见的髓外硬膜下肿瘤,更常见于女性。多发性的脊膜瘤可能也伴有神经纤维瘤病。不同于许旺氏细胞瘤,脊膜瘤主要位于上段和中段胸椎水平,但是在前后方向有相似的分布。神经鞘瘤和脊膜瘤都可以累及硬膜内外,而表现为哑铃状。

MRI is the modality of choice in the evaluation of intradural-extramedullary spinal tumors. Nerve sheath tumors and meningiomas demonstrate characteristics of craniocaudal location divergence, hyperintensity and heterogeneity of T2W images, intensity and heterogeneity of enhancement, and the presence or absence of the “dural tail sign”. Schwannomas are typically hyperintense and heterogeneous compared to meningiomas on T2W images. The tumors show different contrast enhancement- meningiomas enhance moderately and homogenously, while schwannomas enhance strongly and irregularly. The “dural tail sign,” a reactive thickening of dura tapering away from the tumor, is associated with meningiomas, though this is not a specific sign. Additionally, neural foraminal extension and foraminal widening is suggestive of schwannoma, while bony sclerosis surrounding the mass is suggestive of meningioma.

对于髓外硬膜下肿瘤,MRI是首选的检查方式,神经鞘瘤和脊膜瘤显示的特征性表现可以帮助我们建立一个鉴别诊断体系,这居于病变的部位、T2序列上信号的强度和均匀性、强化程度和均匀性以及是否有脑膜尾征。许旺氏细胞瘤与脊膜瘤相比,通常信号更高更不均匀;两者的强化程度也不同,脊膜瘤中度均匀性强化,而许旺氏细胞瘤明显强化而不规则。脑膜尾征,即反应性的硬脊膜增厚,常常见于脊膜瘤,虽然这不是一个特异性的征像。另外,神经孔的扩大和增宽也提示神经鞘瘤,而环绕肿瘤的骨质硬化提示脊膜瘤。

The differential diagnosis for intradural-extramedullary spinal mass lesions includes nerve sheath tumor, meningioma, metastasis, paraganglioma, vascular malformations, inflammatory process, and developmental lesions. Vascular tumors may necessitate pre-operative angiography and embolization

脊椎髓外硬膜下肿块的鉴别诊断包括神经鞘瘤、脊膜瘤、转移瘤、副神经节瘤、血管畸形、炎性病变以及发育性病变。血管性肿瘤可能需要采取术前的血管造影和栓塞。

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