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室管膜下巨细胞型星形细胞瘤 被引量:7

Subependymal giant cell astrocytoma
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摘要 目的探讨室管膜下巨细胞型星形细胞瘤的病理、临床表现、影像学(CT、MRI)、诊断及治疗,以便提高诊断率和治愈率。方法回顾分析自1996年9月至2004年8月收集的12例室管膜下巨细胞型星形细胞瘤的临床特点,头颅CT、MRI及病理学特征。结果(1)室管膜下巨细胞型星形细胞瘤好发于青少年儿童,男性多于女性,以"三联征"和不同程度神经系统缺失为特征的首发临床表现。本组12例中5例伴发结节性硬化综合征。(2)影像学多数病例头颅CT表现侧脑室室管膜下或脑室内门氏孔附近呈椭圆形或不规则结节状较均匀低密度肿块,少数为等或高密度,伴室管膜下多发点状钙化。MRI常表现等T1、等T2,少数出现T1高信号、T2低信号。病变周围没有或很少水肿。CT或MRI增强扫描病变出现均匀强化。(3)临床诊断应与室管膜下室管膜瘤、室管膜下结节、室管膜瘤及脉络丛乳头状瘤等相鉴别。(4)只要全切除,术后不必放、化疗。结论典型病例较易诊断,绝大多数室管膜下巨细胞型星形细胞瘤为WHOⅠ级,只要全切除预后良好。 Objective To analyze the clinical characteristics, neuroradiological findings, pathology and treatment of patients with subependymal giant cell astrocytoma (SEGA). Methods A series of 12 patients with SEGA were reviewed between September 1996 and August 2004. The pathology, clinical presentations, CT and MRI, diagnosis, treatment and pmguosis of patients with SEGA were analyzed. Resttlts ( 1 ) The tumor typically affects teenager, and there was a male predominance ( male-to-female ratio = 3:1 ). "epiloia" and neurologic deficiency was the first presentations. Five of 12 cases with SEGA were concomitant of tuberous sclerosis complex. (2) Imageology CT characteristically demonstrated a oval or irregular type nodular heterogeneity hypoindense lesion in the region of the subependymal membrane or nearby Monro's formnen in the most Patients with SEGA, but presented as iseindense or hyperindense a few cases, which usually accompanied with multiple poits or nodular calcifications in subependymal membrane. MRI shown that isointense on T1 weighted and T2 weighted in the majority Patients, but a few cases as hyperintense on T1 weighted and hypointense on T2 weighted . The lesion surrounding no or a few vasogenic edema was found. The heterogeneity contrast-enhancing lesions shown on CT or MRI in all the patients with SEGA. ( 3 ) It was very important to differentiate SEGA from subependymoma, subependymal nodular, epedymoma and choroid plexus papiloma. (4) If the lesion was completely removed by surgery, the radiotherapy and chemotherapy were not necessary after operation, prognosis was satisfied. Conclusion Majority of SEGA belongs to a specific benign tumor of the brain. It is easier to diaguosis as astrocytoma type I, which is associated with a favorable clinical outcome, especially if completely excised.
出处 《中华神经外科杂志》 CSCD 北大核心 2007年第5期376-379,共4页 Chinese Journal of Neurosurgery
关键词 星形细胞瘤 室管膜下巨细胞 计算机断层扫描 磁共振成像 Astrocytoma, Subependymal giant cell CT MRI
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  • 2Yamamoto K,Yamada K,Nakahara T,et al.Rapid regrowth of solitary subependymal giant cell astrocytoma-case report[J].Neurol Med Chir (Tokyo), 2002,42(5):224-227. 被引量:1
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