Clinical features and prognosis for intraventricular liponeurocytoma.

Nini Xu,Peiyi Gao,Jian Zhou,Jiang Du,Huachen Zhu,Xiaofeng Li,Rong Yang,Jinxiu Cai

doi:10.18632/oncotarget.16024

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.

Highlights

Cerebellar liponeurocytoma is a very rare neoplasm of adults with distinctive morphologic feature of central nervous system (CNS)
Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor
The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on Computed tomography (CT) scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI)

Summary

Introduction

Cerebellar liponeurocytoma is a very rare neoplasm of adults with distinctive morphologic feature of central nervous system (CNS). In the 2007 WHO classification, the tumor was defined as WHO grade II for its low proliferation, but high likelihood of recurrence, and the definition is still in use up to now [3, 4]. This neoplasm develops predominantly in cerebellar hemisphere and vermis, occasionally in supratentorial lateral ventricle and the fourth ventricle. Some reports indicated that patients with cerebellar liponeurocytomas usually show a favorable clinical course after neurosurgical removal, it means that the accurate diagnosis of this tumor is very important to avoid unnecessary adjuvant therapy

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Results

Conclusion