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Cerebellar liponeurocytomas, also known as neurolipocytomas, are rare tumors of the cerebellum with neurocytic differentiation and abundant intracellular lipid. They are considered WHO grade 2 tumors 1.
These tumors have been reported at a wide range of ages (4-77 years) but seem to be encountered typically in adults of middle age 1,2. There is no strong gender predilection 1,2.
The clinical presentation is non-specific, relating to raised intracranial pressure (e.g. headache) and cerebellar signs (e.g. ataxia) 1.
As the name implies, the vast majority of cases of liponeurocytoma arise in the cerebellum 2. However, up to a fifth of reported cases have been supratentorial, around or within the ventricular system 2.
They are designated as WHO grade 2 tumors and considered one of the glioneuronal and neuronal tumors in the WHO classification of central nervous system tumors 1,2,4.
These tumors are composed of small neurocytic cells and neuroepithelial cells with abundant cytoplasmic lipid 1.
Cerebellar liponeurocytomas appear as heterogeneous intra-axial or, uncommonly, intraventricular masses 2. The most common location is in the cerebellar hemispheres.
The tumor is usually hypodense but occasionally isodense to surrounding parenchyma 2.
- T1: more commonly hypointense, sometimes isointense, only occasionally hyperintense to suggest macroscopic fat (18%) 2
- T2/FLAIR: usually hyperintense 2
- T1 C+: heterogeneous enhancement 1,2
Treatment and prognosis
Cerebellar liponeurocytomas can be cured with complete resection and adjuvant radiotherapy 2. However, recurrence is common with incomplete resection followed by radiotherapy (17%) or with complete resection without adjuvant radiotherapy (26%) 2.
- 1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
- 2. Gembruch O, Junker A, Mönninghoff C, Ahmadipour Y, Darkwah Oppong M, Sure U, El Hindy N, Lemonas E. Liponeurocytoma: Systematic Review of a Rare Entity. (2018) World neurosurgery. 120: 214-233. doi:10.1016/j.wneu.2018.09.001 - Pubmed
- 3. Takami H, Mukasa A, Ikemura M, Shibahara J, Takahashi M, Momose T, Saito N. Findings from positron emission tomography and genetic analyses for cerebellar liponeurocytoma. (2015) Brain tumor pathology. 32 (3): 210-5. doi:10.1007/s10014-014-0210-4 - Pubmed
- 4. Louis D, Perry A, Wesseling P et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro-Oncology. 2021;23(8):1231-51. doi:10.1093/neuonc/noab106 - Pubmed