病史
男，50岁，双手麻木乏力5年，左手加重1年余。
上期试题：血管母细胞瘤可合并D
A、神经纤维瘤病1型
B、结节性硬化
C、Chiari畸形
D、Von Hippel-Lindau病
E、神经纤维瘤病2型
男，50岁，双手麻木乏力5年，左手加重1年余。
室管膜瘤(ependenoma)是最常见的髓内肿瘤，约占60％，起源于中央管的室管膜细胞或终丝等部位的室管膜残留物，可发生于脊髓任何节段，多见于腰骶段、脊髓圆锥和终丝。最常见于成年人，发病高峰在40～50岁，男性略多于女性。肿瘤生长缓慢，临床病程时间长，临床症状无特异性，主要取决于与肿瘤的大小、部位。
Ependymoma (ependenoma) is the most common type of intramedullary tumor, accounting for about 60%, originated in the central tube of ependymal cells or in areas such as the filum terminale ependymal residue, can happen at any segmental spinal cord, see more at lumbosacral segment, conus medullaris and filum terminale. It is most common in adults, with a peak of 40 to 50 years, and slightly more men than women. The tumor growth is slow, the clinical course is long, the clinical symptoms are not specific, mainly depends on the size and location of the tumor.
由于室管膜瘤起源于中央管表面室管膜细胞，瘤体常位于脊髓中央，沿脊髓长轴呈中心性纵行生长；室管膜瘤的另一特征性表现是肿瘤边界清。
Since ependymoma originates from ependymoma cells on the surface of the central canal, the tumor is often located in the center of the spinal cord and grows medially along the long axis of the spinal cord. Another characteristic of ependymoma is clear tumor boundary.
WH02000年所颁布中枢神经系统肿瘤分类将室管膜肿瘤分为：粘液乳头状室管膜瘤、室管膜下瘤、室管膜瘤及间变性室管膜瘤，室管膜瘤又分为细胞型、乳头型、透明细胞型及脑室膜细胞4个亚型。肿瘤分级：粘液乳头状室管膜瘤、室管膜下瘤为I级，室管膜瘤为Ⅱ级，间变性室管膜瘤为Ⅲ级。发生于颈髓多为细胞型或混合型室管膜瘤，发生于马尾终丝多为粘液乳头状型。
WH02000 years promulgated by the central nervous system tumor classification ependymal tumors can be divided into: mucus papillary ependymoma, subependymal tumor, ependymoma and anaplastic ependymoma, ependymoma is divided into cells, nipple, transparent cell type and ependyma cells four subtypes. Staging: mucus papillary ependymoma, subependymal tumor for grade I, ependymoma to Ⅱ anaplastic ependymoma Ⅲ level. The cervical medulla is mostly cell type or mixed type ependymoma, and the cauda equina is mostly mucinous papillary type.
在T1WI多呈等、低信号，在T2WI多呈高信号，若肿瘤合并出血或囊变，呈混杂信号。根据出血时间的不同，血肿信号表现复杂，陈旧性的瘤内出血伴囊变，可见分层液平，肿瘤表面的陈旧性出血，由于含铁血黄素的沉积，在T2WI表现为低信号环，即“帽征“。囊变是造成肿瘤信号不均的另一原因，与脊髓室管膜瘤相关的囊变分为三种：
In T1WI, the signal is mostly equal and low, while in T2WI, the signal is mostly high. If the tumor is complicated with bleeding or cystic degeneration, the signal is mixed. Depending on the bleeding time, hematoma complicated signal performance, pulmonary tumor hemorrhage with cystic change, visible stratified fluid, pulmonary hemorrhage, on the surface of the tumor with hemosiderin deposition, for low signal in T2WI manifestation, namely 'cap'. Cystic degeneration is another cause of tumor signal imbalance. Cystic degeneration associated with ependymoma of spinal cord can be divided into three types:
①瘤内囊，为肿瘤囊变，囊壁由肿瘤细胞构成，增强后囊壁强化，手术时必须切除；
1、The cyst inside the tumor is the tumor cyst change, and the cyst wall is composed of tumor cells.
②肿瘤头、尾端的囊腔，是周围脊髓组织对肿瘤的反应性改变．形成机制包括正常脊髓的缺血造成的脊髓软化、肿瘤坏死或出血的降解产物在肿瘤两端的聚集，导致高渗性囊腔的形成，囊液为黄色或血性液体，囊壁有正常的胶质细胞，增强后囊壁不强化；
2、The cystic cavity of the tumor's head and tail is the reactivity change of surrounding spinal tissue to tumor. Formation mechanism including the normal spinal cord ischemia caused by spinal cord softening, tumor necrosis or hemorrhage at the ends of the degradation products in tumor gathered themselves together, and leads to the lumen of the high permeability formation, fluid for yellow or bloody fluid, the capsule wall have normal glial cells, cystic wall not enhancement;
③脊髓中央管的扩张，由于肿瘤堵塞了脑脊液的正常循环通路所形成，囊液为脑脊液。/3、The expansion of the central canal of the spinal cord is due to tumor blocking the normal circulation pathway of cerebrospinal fluid, which is cerebrospinal fluid.
脊髓室管膜瘤为富血供肿瘤，明显的、边界清楚的均匀强化是其又一特点。
Ependymoma of spinal cord is a tumor rich in blood supply.
1、 星形细胞瘤：占髓内肿瘤的25％，多见于儿童，以颈胸段最为常见，一般呈浸润性、偏心性生长。T1WI呈低信号，T2wI呈明显高信号，病变可出血、坏死、囊变，信号常不均匀，坏死、囊变部分呈更长T1WI和更长T2WI信号。增强扫描一般呈不均匀性轻中度强化。星形细胞瘤与正常脊髓组织分界不清，手术难以完全切除，预后较差。而室管膜瘤多发生于年轻人，可发生于脊髓各段，以腰骶段、脊髓圆锥及终丝较常见，肿瘤呈中心性生长。增强后明显强化，边界清楚，手术易于完全切除，预后较好。
1. Astrocytoma: accounting for 25% of intramedullary tumors, most commonly seen in children, most common in the cervicothoracic segment, generally with invasive and eccentrical growth. T1WI showed low signal, T2wI showed obvious high signal, and the lesions could be bleeding, necrosis and cystic degeneration. The signal was often uneven, and the necrotic and cystic lesions showed longer T1WI and longer T2wI signals. The enhanced scanning is generally non-uniform mild to moderate enhancement. The boundary between astrocytoma and normal spinal tissue is not clear, so it is difficult to completely remove the tumor after surgery, and the prognosis is poor. In contrast, ependymoma mostly occurs in young people, and can occur in all segments of the spinal cord. Lumbosacral segment, spinal cone and terminal filament are more common, and tumor growth is centripetal. After enhancement, obvious enhancement, clear boundary, easy complete resection, good prognosis.
2、血管母细胞瘤：脊髓内血管母细胞瘤较少见，位于髓内的血管母细胞瘤也可表现为脊髓的弥漫性增粗、囊变、出血，应与室管膜瘤鉴别。脊髓内血管母细胞瘤占髓内肿瘤的3％～8％，多见于40岁左右患者，儿童少见；血管母细胞瘤属于良性肿瘤，起源不明，无包膜，但与脊髓界限清楚；可发生于脊髓任何节段，以颈段脊髓最为常见，多生长在脊髓内背侧或背外侧；肿瘤在T1wI上呈等或高信号，在T2wI上为高信号，肿瘤内可见流空血管信号影，为其重要特征。另外，有些脊髓血管母细胞瘤也表现出典型颅内血管母细胞瘤大囊小壁结节强化的特点，有助于血管母细胞瘤的诊断。
2, vascular tumor, vascular tumor is rare in the spinal cord, located in intramedullary tumor blood vessels can be characterized by diffuse enlargement of the spinal cord, cystic change, bleeding, should with ependymoma. Intramedullary angioblastoma of spinal cord accounts for 3% ~ 8% of intramedullary tumors, mostly in patients around 40 years old, rarely seen in children. Angioblastoma is a benign tumor with unknown origin and no capsule, but it has a clear boundary with the spinal cord. It can occur in any segment of the spinal cord, most commonly in the cervical spinal cord. Tumor showed equal or high signal on T1wI, high signal on T2wI, and empty blood vessel signal shadow was seen in tumor, which was an important feature. In addition, some spinal hemangioblastoma also showed the characteristics of the enhancement of the small wall nodules in the large sacs of typical intracranial hemangioblastoma, which is helpful for the diagnosis of angioblastoma.
3、转移瘤：髓内转移瘤与室管膜瘤鉴别主要依据是脊髓增粗不明显，脊髓囊变较少见，而且多有原发灶。
3. Metastatic tumor: the differentiation between intramedullary metastases and ependymoma is mainly based on the inconspicuous thickening of the spinal cord, the rare cystic degeneration of the spinal cord, and the primary lesion.
本期试题：室管膜瘤最好发于？
A . 第三脑室
B . 第四脑室
C . 侧脑室
D . 中脑导水管
E . 脊髓中央管
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