Xu Nini, Cai Jinxiu, Du Jiang, Yang Rong, Zhu Huachen, Gao Peiyi, Zhou Jian, Li Xiaofeng
Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
Oncotarget. 2017 Mar 8;8(37):62641-62647. doi: 10.18632/oncotarget.16024. eCollection 2017 Sep 22.
Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor. We retrospectively analyzed the clinical, radiological and histopathological findings as well as follow-up data of two patients with intraventricular liponeurocytomas in Beijing Tiantan Hospital between July 2000 and July 2016. The main clinical manifestations of the two patients were headache. The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on CT scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI). The plaque-like hypodense on CT images and hyperintensity on T1WI resembling fat could be seen inside the tumor. The liponeurocytoma located in the fourth ventricle showed isointensity on T1 and T2WI as well as slight enhancement on contrast. Two patients accepted gross total resection of tumors. Two intraventricular tumors demonstrated similarly histopathological features, such as isomorphic small tumor cells with clear cytoplasm, sheets of monomorphic round cells and focal lipomatous differentiation. In addition, expression of synaptophysin, neuron specific enolase, microtubule-associated protein 2 and S-100 were found. No radiological or clinical evidence of recurrence of the tumors was observed in their follow-up surveys. In conclusion, intraventricular liponeurocytoma has a favorable clinical course, radiological features may be useful in the diagnosis of this rare tumor before surgery.
小脑脂肪神经细胞瘤是一种罕见的中枢神经系统肿瘤，我们对其生物学行为和临床预后进行研究，以增进对该肿瘤的了解。我们回顾性分析了2000年7月至2016年7月在北京天坛医院就诊的2例脑室内脂肪神经细胞瘤患者的临床、影像学和组织病理学表现以及随访数据。2例患者的主要临床表现均为头痛。幕上脑室内脂肪神经细胞瘤在CT扫描上表现为等密度至轻度高密度，在T1加权成像（T1WI）和T2加权成像（T2WI）上呈不均匀信号强度。肿瘤内可见CT图像上的斑片状低密度影和T1WI上类似脂肪的高信号。位于第四脑室的脂肪神经细胞瘤在T1WI和T2WI上呈等信号，增强扫描有轻度强化。2例患者均接受了肿瘤全切术。2例脑室内肿瘤具有相似的组织病理学特征，如胞质清晰的同型小肿瘤细胞、成片的单形圆形细胞以及局灶性脂肪化生。此外，还发现了突触素、神经元特异性烯醇化酶、微管相关蛋白2和S-100的表达。随访期间未观察到肿瘤复发的影像学或临床证据。总之，脑室内脂肪神经细胞瘤临床病程良好，影像学特征可能有助于术前诊断这种罕见肿瘤。
