Liponeurocytoma
see Cerebellar liponeurocytoma.
Cerebellopontine angle liponeurocytoma
see also Ventricular Liponeurocytoma.
Liponeurocytoma is a very rare tumor classified as grade II (Neuronal and mixed neuronal glial tumors) according to the World Health Organization Classification of Tumors of the Central Nervous System 2016. The median age is 50-years-old and the most frequent location is the posterior cranial fossa especially within the cerebellar hemispheres; those arising in the cerebellopontine angle are exceptional 1).
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 by Gembruch et al from the Department of Neurosurgery, Department of Neuropathology and Institute of Diagnostic and Interventional Radiology and Neuroradiology, University HospitalEssen, Germany. 4 Department of Neurosurgery, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
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 2).