Cai Yuxiang, Wang Yanjin, Yang Zhiquan
J Neurosurg Pediatr. 2020 Apr 10;26(1):34-37. doi: 10.3171/2020.2.PEDS19764. Print 2020 Jul 1.
Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracranial tumors of neuroglial origin, mostly located in the supratentorial regions and particularly in the temporal lobe. Few cases of DNETs in the hypothalamus have been described. The authors present the case of a DNET in the hypothalamus. The 5-year-old girl with complaints of limb and gelastic seizures was admitted to the neurosurgical department of Xiangya Hospital. Neurological examination findings were unremarkable. MRI showed isointensity without significant enhancement on T1- and T2-weighted images. The lesion exhibited clearly defined borders on the sagittal, coronal, and axial images. The preliminary diagnosis was hypothalamic hamartoma (HH); however, the lesion was surgically removed, and histopathological examination confirmed the diagnosis of a DNET. Hypothalamic DNETs are extremely rare. Based on their clinical manifestation and imaging, DNETs are easily misdiagnosed as HHs. Diagnoses apart from HHs must be entertained when a hypothalamic lesion is being investigated.
胚胎发育不良性神经上皮肿瘤（DNETs）是起源于神经胶质的良性颅内肿瘤，大多位于幕上区域，尤其是颞叶。下丘脑区域的DNETs病例鲜有报道。本文作者报告了一例下丘脑DNETs病例。一名5岁女童因肢体发作和痴笑样发作入院至湘雅医院神经外科。神经系统检查未发现明显异常。MRI显示在T1加权像和T2加权像上呈等信号，无明显强化。在矢状位、冠状位和轴位图像上，病变边界清晰。初步诊断为下丘脑错构瘤（HH）；然而，该病变经手术切除，组织病理学检查确诊为DNET。下丘脑DNET极其罕见。基于其临床表现和影像学表现，DNET很容易被误诊为HH。在研究下丘脑病变时，必须考虑到除HH之外的其他诊断。
