Cerebellar liponeurocytoma – a rare entity: a case report

Abstract

BackgroundCerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differentiation. Liponeurocytoma develops in adult patients and is defined in the World Health Organization classification of 2016 as a rare benign grade II tumor.Case presentationA 39-year-old Italian man presented to our department suffering from headache and nausea. Magnetic resonance imaging revealed a right-sided cerebellar lesion showing poor contrast enhancement without an obstructive hydrocephalus. Surgery was indicated and total tumor resection was achieved. He was discharged without any neurological deficits. Histopathological examinations revealed a cerebellar liponeurocytoma. A neurological follow-up examination revealed no neurological deficit directly after surgery and 1 year later. Radiotherapy was recommended at the neurooncological board despite the total removal of the tumor, but our patient refused adjuvant radiotherapy. Magnetic resonance imaging of his neurocranium with and without contrast enhancement 48 hours after surgery and 15 months after surgery showed no residual tumor.ConclusionsLiponeurocytomas are rare benign tumors occurring in the majority of cases in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy has to be discussed individually, but seems to be sufficient if complete tumor resection is not achieved or in cases of a tumor recurrence.

Cerebellar Liponeurocytoma: A Rare Fatty Tumor and its Literature Review

“…[8] Recent reports also exist in the form of individual cases, the most common being in the infratentorial location. [910111213] Gembruch et al ., in a systematic review of all the reported cases ( n = 73) until 2018, noted that these tumors had low MIB-1 proliferation index (3.73 ± 4.01%). [14] Of the infratentorial location, the cerebellar hemisphere is the most common location.…”

Cerebellar liponeurocytoma, a rare tumor: Case report and review of the literature

“…A liponeurocytoma is a neurocytic neoplasm that involves a variable extent of neuronal differentiation in addition to a focal lipomatous and astrocytic differentiation that can be seen due to a consistent immunoreactivity to synaptophysin, neuron-specific enolase (NSE) and MAP-2. In addition, the tumor cells may be positive for nuclear NeuN but negative for cytoplasmic IDH1- R132H, epithelial membrane antigen (EMA), cytokeratin (CK), and nuclear Olig-2 [ 8 , 10 ].…”

“…In the lipomatous cells, the lipid vacuole membranes were positive for SYN, MAP-2, vimentin and S-100 and the expression of GFAP and S-100 is limited to scattered reactive astrocytes [ 1 , 8 , 13 ].…”

“…With all this symptomatology, there is some differential diagnosis that we should think about and they include oligodendrogliomas, clear cell ependymomas and high-grade tumors like medulloblastomas. It is easy to misdiagnose a liponeurocytoma with these other differential diagnoses, but we can differentiate them [ 8 , 15 , 16 ].…”

“…Although findings from computed tomography (CT) and magnetic resonance imaging (MRI), and pathological and immunohistochemical characteristics have been reported in around 70 cases, clinical and pathological diagnosis of liponeurocytoma remained challenging and the characteristics of this tumor entity are still poorly understood [ 8 ].…”