Supratentorial intracerebral cerebellar liponeurocytoma

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.