Rationale:Cerebellar liponeurocytoma is a rare tumor of the central nervous system (CNS) characterized by low proliferation but high likelihood of recurrence. Because of its rarity and the paucity of systematic follow-up, the biological behaviors and clinical features of this tumor are still poorly understood. We herein reported a case of cerebellar liponeurocytoma originating in the cerebral hemisphere.Patient concerns:A 11-year-old male with intermittent headache, nausea, and vomiting. The first computed tomography revealed a large mass in the right cerebral hemisphere. He was transferred to our institution for neurosurgical treatment.Diagnosis:Magnetic resonance imaging showed a large cystic—solid mass in the right frontal lobe with obvious contrast enhancement. Histopathological examinations showed sheets of isomorphic small neoplastic cells with clear cytoplasm and focal lipomatous differentiation. On immunohistochemistry, tumor cells were positive for synaptophysin, microtubule-associated protein 2, and neuronal nuclei antigen.Interventions:The patient was performed a right fronto-parietal craniotomy, and gross total resection of the tumor was achieved without adjuvant therapy.Outcomes:No clinical or neuroradiological evidence of recurrence or residual of the tumor was found 6 years and 2 months after initial surgery.Lessons:Cerebellar liponeurocytoma developing in supratentorial cerebral hemisphere was first reported in the present study. The radiological and histopathological features may be useful in differentiating this rare tumor from other tumors at similar locations. A change in the nomenclature of cerebellar liponeurocytomas should be considered in future World Health Organization (WHO) classifications.

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.