Clinical features and prognosis for intraventricular liponeurocytoma

Abstract

// Nini Xu 1 , Jinxiu Cai 1 , Jiang Du 2 , Rong Yang 1 , Huachen Zhu 1 , Peiyi Gao 1 , Jian Zhou 1 and Xiaofeng Li 3,4 1 Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China 2 Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China 3 PET/CT/MRI Center, The Fourth Affiliated Hospital of Harbin Medical University, Harbin, China 4 Department of Radiology, University of Louisville, Louisville, KY, USA Correspondence to: Jian Zhou, email: // Keywords : cerebellar liponeurocytoma; intraventricular; computed tomography; magnetic resonance imaging; immunohistochemistry Received : December 20, 2016 Accepted : February 23, 2017 Published : March 08, 2017 Abstract 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.