CNS tumor
Neuronal and mixed neuronal - glial tumors
Cerebellar liponeurocytoma

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 30 August 2017, last major update March 2015

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Cerebellar liponeurocytoma"

Cite this page: Abdelzaher, E. Cerebellar liponeurocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorcerebellarliponeurocytoma.html. Accessed September 24th, 2017.
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
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+)
Microscopic (histologic) images

Images hosted on other servers:

Oligodendrocyte-like and adipocyte-like cells

Various images

Synaptophysin

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