Neurosurgery notes/Tumours/Gliomas, Glioneuronal Tumors, and Neuronal Tumors/Glioneuronal and neuronal tumours/Cerebellar liponeurocytoma

Cerebellar liponeurocytoma

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Glioneuronal and neuronal tumours

Definition

  • Essential:
    • Cerebellar localization AND
    • Oligodendroglioma-like monomorphic cells associated with focal lipoma-like changes AND
    • Synaptophysin expression AND (for unresolved lesions)
      • Methylation profile of cerebellar liponeurocytoma
  • Desirable:
    • Adult patient Focal GFAP immunoreactivity absence of histological features of malignancy

WHO grading

  • Grade 2
  • Time to clinical progression is often long (mean: 6.5 years)

Number

  • Mean age 50yrs
  • Male: female 1:1
  • 40 cases published

Clinical features

  • Obstructive HCP: H/A
  • Cerebellar signs, including ataxia and disturbed gait, are also common

Localisation

  • Post fossa
    • Cerebellar hemispheres
    • Other paramedian region or vermis
      • Extend to the cerebellopontine angle or fourth ventricle

Origin

  • Cerebellar progenitors, which are distinct from cerebellar granule progenitors
  • Not a variant of medulloblastoma

Radiology

CT

  • Tumour is variably isodense or hypodense, with focal areas of marked hypoattenuation corresponding to fat density

MRI

  • T1: Iso/hypointense with patchy areas of hyperintensity corresponding to regions of high lipid content
  • T1+C: Heterogenous enhancement
  • T2: tumour is slightly hyperintense to the adjacent brain, with focal areas of marked hyperintensity.
    • Little to no oedema
      •  
Fig. 6.47 Cerebellar liponeurocytoma. A Tl -weighted MRI (with gadolinium) of a recurrent liponeurocytoma, presenting as an irregular, strongly enhancing lesion in the right cerebellar hemisphere. Reprinted from Jenkinson MD et al. {1153}. B Axial T I-weighted MRI after gadolinium administration shows areas of prominent hypointense signal within a well- demarcated isodense tumour {53}.
Cerebellar liponeurocytoma.
A, T1-weighted MRI (with gadolinium) of a recurrent liponeurocytoma, presenting as an irregular, strongly enhancing lesion in the right cerebellar hemisphere.
B, Axial T1-weighted MRI after gadolinium administration shows areas of prominent hypointense signal within a well-demarcated isodense tumour

Histopathology

  • Macroscopic
  • Microscopic
      • Composed of small neurocytic cells and lipoma-like lipid-laden cells which represent neuroepithelial cells with abundant cytoplasmic lipid
      • Features of anaplasia such as nuclear atypia, necrosis, and microvascular proliferation are typically absent in primary lesions, but may be found in recurrent tumours
      Fig. 6.48 Cerebellar liponeurocytoma. A Typical histology of cerebellar liponeurocytoma with focal accumulation of adipocytic tumour cells on a background of densely packed small round neoplastic cells, which often show a perinuclear halo. B Note the typical focal lipomatous differentiation of tumour cells, with displacementof nuclei to the cell periphery.
      Cerebellar liponeurocytoma. A, Typical histology of cerebellar liponeurocytoma with focal accumulation of adipocytic tumour cells on a background of densely packed small round neoplastic cells, which often show a perinuclear halo. B, Note the typical focal lipomatous differentiation of tumour cells, with displacement of nuclei to the cell periphery.
  • Immunophenotype
    • Synaptophysin: positive
    • Neurone-specific enolase: positive
    • MAP2: positive
    • GFAP: positive
  • Ki67<3% but can be as high as 6%

Genetic profile

  • BRAF and IDH mutations were absent
  • Losses of chromosomes 14 and 2p

Management

  • Surgery is considered the treatment of choice
  • No real role for radiotherapy

Prognosis

  • Chakraborti et al., 2011
    • 5 yrs. survival rate of only 48%
  • Recurrence and radioresistance can occur

Differential diagnosis

Features
Cerebellar liponeurocytoma
Medulloblastoma
Isochromosome 17q
mutations of PTCH1, CTNNB1, and APC
Absent
Present
TP53 missense mutations
Low rate
Higher rate
Loss of chromosomes 14 and 2p
Present
Absent