CNS tumor
Neuronal and mixed neuronal-glial tumors
Cerebellar liponeurocytoma
Author: Eman Abdelzaher, M.D., Ph.D.
Topic Completed: 1 March 2015
Minor changes: 24 July 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: "Cerebellar liponeurocytoma"
Table of Contents
Definition / general | Epidemiology | Sites | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Microscopic (histologic) description | Positive stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Abdelzaher E. Cerebellar liponeurocytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorcerebellarliponeurocytoma.html. Accessed August 6th, 2020.
Definition / general
- Well differentiated neurocytic neoplasm composed principally of neurocytes admixed with lipidized cells
- Corresponds to WHO grade II
- Historic names: lipomatous medulloblastoma, neurolipocytoma, medullocytoma, lipomatous glioneurocytoma
- First reported in 1978 (Acta Neuropathol 1978;41:261)
- Considered a mixed neuronal - glial tumor; lipid is apparently due to tumoral lipidization, not adipose metaplasia (Am J Surg Pathol 2001;25:1551)
Epidemiology
- Rare neoplasm
- Age: adults (range: 24 - 77, mean age: 50 years)
- Sex: no gender predilection
Sites
- Almost always in cerebellum (usually hemispheric, less commonly vermian or cerebellopontine angle)
- Some cases reported in supratentorial ventricular system but unclear if same entity (Indian J Neurosurg 2012;1:83)
Clinical features
- Headache, cerebellar signs
Radiology description
- Well circumscribed
- Hyperintense on pre contrast T1 weighted images due to lipid content
- Heterogeneous contrast enhancement
- ± Minimal edema
Radiology images
Images hosted on other servers:
Right cerebellopontine angle mass
Right cerebellar hemisphere mass
Prognostic factors
- Overall favorable clinical outcome with no features of malignant progression but frequent recurrence (50%)
- Prognosis may be less favorable if mitotic figures present and > 10% proliferative index (Neuropathology 2005;25:77) although even cases with low mitotic index may progress (J Neurooncol 2005;71:53)
Case reports
- 6 year old girl whose tumor had high labeling index (Hum Pathol 2002;33:564)
- 40 year old woman (Can J Surg 2009;52:E117)
- 49 year old man with cerebellar liponeurocytoma with unusually aggressive histopathology (J Korean Neurosurg Soc 2012;52:250)
- 51 year old woman with recurrent tumor with leptomeningeal invasion but no other aggressive histologic features (Neurosurgery 2003;53:1425)
- 53 year old woman (Arq Neuropsiquiatr 2002;60:725)
- 62 year old woman with liponeurocytoma of the cerebellum (Neurol India 2003;51:274)
Microscopic (histologic) description
- Biphasic with admixture of neurocytes and lipidized cells
- Neurocytes are arranged in densely cellular sheets of monotonous cells with scantty and often clear cytoplasm, rounded to oval nuclei, salt and pepper chromatin
- Lipidized cells are typically clustered and resemble mature adipocytes
- Glial differentiation in some cases in the form of looser and more infiltrative well differentiated cells
- No / low mitotic activity
- Rarely myogenic differentiation (desmin+)
Positive stains
- Neurocytic component: diffusely positive for neuronal markers synaptophysin, NSE and MAP2
- Focal GFAP+ in glial component and reactive astrocytes
- Lipidized cells: neuronal markers+, GFAP+
- Low proliferation index (Ki67), generally < 6%
Electron microscopy description
- Neurocytes: microtubules and neurosecretory granules
- Lipidized cells: contain nonmembrane bound lipid
Differential diagnosis
- Clear cell ependymoma: rare in posterior fossa, perivascular
- Medulloblastoma: children, atypia, high proliferation index, no lipidized cells
- Oligodendroglioma: rare in posterior fossa, no neurocytic component, no lipidized cells
- RGNT: neurocytic rosettes, no lipidized cells
Additional references
