罕少见脑胚胎性肿瘤患者的临床病理特征及其影像表现

Clinicopathologic features and imaging findings of rare and uncommon brain embryonal tumours

目的探讨几种罕少见的脑胚胎性肿瘤的临床病理特征及影像表现。方法回顾性分析10例罕少见的经手术病理证实为脑胚胎性肿瘤患者的临床病理特征及MRI、CT表现。结果10例中，幕上原始神经外胚层肿瘤6例，室管膜母细胞瘤2例，髓上皮瘤1例，非典型性畸胎样或横纹肌样瘤1例，其中，4例为婴幼儿和儿童。各肿瘤的病理组织学表现具有一定特征性，是确诊的依据。肿瘤均位于幕上，体积较大，形态多不规则，呈分叶状，信号、密度不均匀，坏死囊变多见；肿瘤实质CT平扫多呈稍高密度，MRI表现为T2WI呈等或较高信号，T1WI呈较低信号，增强扫描显著强化。结论罕少见脑胚胎性肿瘤患者的MRI、CT表现无明显特异性，结合临床及病理组织学特征对其进行全面认识了解十分必要。

Objective To investigate the clinicopathologic features and imaging findings of several kinds of rare and uncommon brain embryonal tumours. Methods MRI or CT appearance and clinicopathologic features in 10 cases of histopathology confirmed brain embryonal tumours were retrospectively analyzed. Results Among 10 cases, there were supratentorial primitive neuroectodermal tumour （sPNET） in 6, ependymoblastoma in 2, medulloepithelioma and atypical teratoid/rhabdoid tumour （AT/RT） in 1 respectively. Patients in 4 cases were infancy and childhood. There was characteristic of the clinicopathologic features of tumors that were the evidence of accurate diagnosis. All lesions were located in the supratentorial region. The tumors were large, usually lobulated in shape irregularly and showed heterogeneous density or signal intensity with some cystic and necrotic areas. The tumors parenchyma appeared slightly high density on CT imaging, iso- or high signal on T2WI and slight low signal on T1WI with intense enhancement after contrast injection. Conclusion MRI or CT findings of rare and uncommon brain embryonal tumours were not characteristic, it was very essential to know the tumors combined with the clinical and pathologic features.