++胚胎发育不良性神经上皮瘤

讨论
• The imaging appearance of dysembryoplastic neuroepithelial tumor is similar to those of other lowgrade glial tumors. On CT, a wedge shaped low-density area is seen in a cortical/ subcortical location “pointing” towards the ventricle. Calcification may be seen in 25% of cases. Scalloping of the adjacent inner table of the skull suggests long standing nature of the lesion. • • DNET影像表现类似于其他的低级别胶质瘤。在CT上，表 现为皮质或皮质下楔形的病变，尖端指向脑室。钙化可以 出现在25％的病例中。邻近的颅骨内部呈扇贝样改变，提 示病变的长期性。
要点
• DNET is a well-demarcated, wedge based “bubbly” intracortical mass in young patients with longstanding seizures. • DNET是一种边界清楚、楔形囊泡状皮质内肿块， DNET 见于年轻患者，多伴有长期的癫痫。 • The temporal lobe (often amygdala / hippocampus) is the most common site. • 颞叶（常常是杏仁核和海马区）是最常见的部位。
讨论ห้องสมุดไป่ตู้
• DNET is an uncommon benign intracortical lesion that is classified by WHO as a “neuronal and mixed neuronoglial tumor”. These are commonly associated with cortical dysplasia. DNETs show no or very slow growth over time and are WHO Grade I. • DNET是一种不常见的良性皮质内病变，WHO分类为“神 经元和混合性神经胶质肿瘤”。常常伴有皮质发育不良。 DNETs显示为无或非常缓慢的生长，WHO分级为I级。
• 预后良好，甚至不完全切除都会有较长的生存期。大多数 病人的癫痫发作有明显减少。肿瘤复发极少见。
讨论
• These tumors virtually always manifest in patients with medically refractory partial seizures. The vast majority of patients are younger than 20 years, and males are more commonly affected. The temporal lobe is the most common site (62%), followed by the frontal lobe (31%). • • 患者通常表现为医学上难治性的部分癫痫发作。绝大多数 患者小于20岁，男性多见。颞叶是最常见的发病部位（62 ％），其次为额叶。
讨 论
• The prognosis is excellent with long survival even with incomplete tumor resection. Most patients have a significant reduction in seizure frequency. Tumor recurrence is very rare.
胚胎发育不良性 神经上皮瘤 (DNET) Dysembryoplastic Neuroepithelial tumor
病史
• A 17-year-old male presents with history of intractable seizures. • 17岁男性出现顽固性癫痫。
• 诊断： 胚胎发育不良性神经上皮瘤
讨论
• At MR imaging, DNET tumors most commonly manifest as cortical masses that are hypointense on T1-weighted images and hyperintense on T2-weighted images without surrounding vasogenic edema. Pseudocystic, multinodular “bubbly” mass may be seen. DNETs usually do not enhance- faint focal punctuate or nodular enhancement may be seen in 20% cases. • 在MR影像上，DNET最常见的表现是皮质内肿块，T1低 信号，T2高信号，无瘤周水肿。假囊状、多结节的囊泡状 肿块，通常表现为无强化－不明显的局部强化或结节状强 化，可见于20％的病例。