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.
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Epidemiology
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.
Clinical presentation
The clinical presentation is non-specific, relating to raised intracranial pressure (e.g. headache) and cerebellar signs (e.g. ataxia) 1.
Pathology
Location
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.
Classification
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.
Microscopic features
These tumors are composed of small neurocytic cells and neuroepithelial cells with abundant cytoplasmic lipid 1.
Immunophenotype
synaptophysin: positive
neuron-specific enolase: positive
MAP2: positive
GFAP: positive
Radiographic features
Cerebellar liponeurocytomas appear as heterogeneous intra-axial or, uncommonly, intraventricular masses 2. The most common location is in the cerebellar hemispheres.
CT
The tumor is usually hypodense but occasionally isodense to surrounding parenchyma 2.
MRI
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
PET
Cerebellar liponeurocytomas demonstrate high C-11 methionine uptake but lower F-18 FDG uptake than normal brain cortex 3.
Treatment and prognosis
Cerebellar liponeurocytomas can be cured with complete resection and adjuvant radiation therapy 2. However, recurrence is common with incomplete resection followed by radiation therapy (17%) or with complete resection without adjuvant radiation therapy (26%) 2.