Liponeurocytoma is a rare benign tumor of the central nervous system, which develops mainly in adult patients within the posterior fossa. The WHO has categorized this entity in their last classification of 2016 as a benign grade II tumor. Histopathological characteristics contain neuronal and variable astrocytic differentiation with foci of lipomatous distinction. Up to now only a few case reports and case series are published and the knowledge of this tumor is still very limited. General treatment guidelines do not exist. Aim of this study was to analyze the literature to create treatment guidelines. PRISMA (i.e., Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to query existing online databases between January 1, 1978 and May 15, 2018. Systematic review of the literature revealed 73 cases (40 female, 33 male) of a liponeurocytoma whereof 59 cases (80.8%) occurred in the posterior fossa. Ki-67/MIB-1 proliferation index was described in 58 cases showing a mean value of 3.73% ± 4.01. Follow-up was performed in 49 cases with a median length of 52.02 ± 50.52 months, showing tumor recurrence in 14 cases (28.57%). Tumor recurrence was observed in only one case (1/12, 8.33%) after adjuvant radiotherapy, while tumor recurrence was seen in 13/29 patients (44.83%), who did not receive adjuvant radiotherapy. Liponeurocytomas are rare benign tumors, occurring mainly in the cerebellum. Therapy of choice is surgery. Postoperative radiotherapy seems to lower the risk of tumor recurrence and should be offered to the patient.
Cerebellar 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. A 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. Liponeurocytomas 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.