目的 分析颅内胚胎发育不良性 神经上皮瘤的影像表现及病理特点，提高 对该类肿瘤诊断的准确性。方法 回顾性 分析经手术病理证实的胚胎发育不良性 神经上皮肿瘤11例，3例行CT检查，其中2 例行CT和MR检查，10例行MR检查，其中7 例行增强扫描，5例加扫波普检查，3例行 PET检查。结果 本组均为单发病灶，位于 幕上10例(颞叶3例，顶叶1例，额叶3例， 岛叶1例、枕叶2例)，小脑半球1例。11例 位于皮层或皮层下，形态呈脑回状5例、 楔形4例、不规则状2例。CT表现：2例呈 低密度，1例呈混杂高密度，其中2例临近 颅骨内板呈稍受压变薄改变。病理组织学 分型：复杂型5例(其中4例见厚薄均匀的 线样分隔征，3例合并壁内结节)，简单型 6例(其中2例见厚薄均匀的线样分隔征)。 8例呈囊样改变，T1WI呈低信号、T2WI呈 高信号，2例T1WI呈混杂低信号，T2WI呈 混杂高信号，FLAIR 8例病灶周围呈环状 高信号，2例呈等低信号。DWI8例均呈等 低信号，2例呈等稍高信号，增强扫描大 部分病灶无强化，壁结节呈轻度强化。2 例病灶内部可见散在小点状强化。9例周 围均无水肿，2例周围可见片状水肿带。5 例波普均表现为胆碱(Cho)峰较对侧正常 脑组织轻度升高，N-乙酰天门冬氨酸(NAA 峰)未见明显降低，3例行PET检查呈低代 谢。结论 胚胎发育不良性神经上皮瘤影 像学表现具有一定的特征性，运用多模态 影像检查方法，可以提高诊断准确性。

Objective To analyze the imaging features and pathological features of intracranial Dysembryoplastic neuroepithelial tumor, so as to improve the diagnostic accuracy of the tumor. Methods 11 cases with pathologically proved DNET were retrospectively reviewed,included 3 cases with CT scan (2 cases with CT and MR scan), 10 patients with MRI scan, 7 cases with enhanced MR scan, 5 cases with MRS scan and 3 cases with 18 F-DPG PET. Results This group were single lesions, located in the supratentorial region and involved the cerebral cortex,including temporal lobe(3 cases), parietal lobe(1 case), frontal lobe (3 cases), insular lobe(1 case), occipital lobe(2 cases), and cerebellar hemisphere (1 case). The lesions showed cerebriform (5 cases),wedge-shaped (5 cases), irregular-shaped (2cases). Hypodensity was seen on CT in all cases, except one of mixed hypo and isodensity, bone thinning of the adjacent calvaria could be seen in 2 cases. Histologically, the DNT were classified into two subtypes：complex form (n=5, 4 cases showed a uniform thickness of wire like separated syndrome, 3 cases with intramural nodule) and simple form (n=6,2 cases showed a uniform thickness of wire like separated syndrome). 8 cases showed cystic changes on MRI. All lesions were hypointense on T1- weighted images and hype rintense on T2-weighted images except two of mixed hypo and isodensity on T1-weighted images and mixed hype and isodensity on T2-weighted images. On T2-FLAIR, 8 cases appeared visible hyperintense ring sign in the tumor periphery, 2 cases appeared mixed hypo and isodensity. On DWI, 8 cases were low signal, 2 cases showed slightly higher signal.Most of the lesions were not enhanced, but the wall nodules were slightly enhanced. 2 cases of internal lesions were scattered in small spot strengthening. There were no edema around all cases except 2 cases. On MR spectroscopy (MRS), The choline (Cho) peak was slightly higher than that of the contralateral normal brain tissue, and there was no significant reduction in N-acetyl aspartate (NAA peak). PET examination showed a low metabolism. Conclusion The imaging features of the intracranial Dysembry oplastic neuroepithelial tumor have certain characteristics, and the diagnostic accuracy can be improved by using the multi-modality imaging method.