CNS & pituitary tumors

Gliomas, glioneuronal tumors, and neuronal tumors

Neuronal and mixed neuronal-glial tumors

Cerebellar liponeurocytoma



Last author update: 2 January 2024
Last staff update: 2 January 2024

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PubMed Search: Cerebellar liponeurocytoma

Davide Mulone, M.D.
Valeria Barresi, M.D., Ph.D.
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Cite this page: Mulone D, Barresi V. Cerebellar liponeurocytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorcerebellarliponeurocytoma.html. Accessed February 22nd, 2024.
Definition / general
  • Cerebellar liponeurocytoma is a rare, slow growing tumor of the central nervous system with advanced neuronal or neurocytic differentiation, variable glial differentiation and lipoma-like changes
  • Classified CNS WHO grade 2
  • Typically occurs in the posterior cranial fossa
  • To date, nearly 70 cases have been reported
Essential features
  • Rare, slow growing tumor with advanced neuronal or neurocytic differentiation and focal lipoma-like changes, typically occurring in the cerebellum
  • Classified as CNS WHO grade 2
  • Extent of surgical resection is the main prognostic factor
  • Histologically composed of monotonous neurocytic cells with rare mitoses and intermingled lipidized cells
  • Diffusely positive for neuronal markers syanptophysin, NeuN and MAP2
Terminology
  • Lipomatous medulloblastoma (not recommended)
  • Lipidized medulloblastoma (not recommended)
  • Medullocytoma (not recommended)
  • Lipomatous glioneurocytoma (not recommended)
  • Lipidized mature neuroectodermal tumor (not recommended)
ICD coding
  • ICD-O: 9506/1 - cerebellar liponeurocytoma
  • ICD-11: 2A00.3 & XH2GB0 - central neurocytoma of brain & cerebellar liponeurocytoma
Epidemiology
Sites
Pathophysiology
  • Supposed to originate from GABAergic neurons in the cerebellar ventricular zone, which may aberrantly differentiate into adipocyte-like tumor cells (J Neurooncol 2012;108:513)
Etiology
Clinical features
Diagnosis
  • Based on imaging (computerized tomography, magnetic resonance) / biopsy / resection specimen
Radiology description
Radiology images

Contributed by Valeria Barresi, M.D., Ph.D.
T1 weighted MRI

T1 weighted MRI

T2 weighted MRI

T2 weighted MRI



Images hosted on other servers:

Right cerebellopontine angle mass

Prognostic factors
Case reports
Gross description
Frozen section description
  • Tumor composed of small, roundish, uniform cells (World Neurosurg 2018;112:18)
  • Infrequent mitoses
  • Microvascular proliferation and necrosis are typically absent
  • Neuropil matrix around vessels may simulate perivascular pseudorosettes of ependymoma
  • Lipid accumulation can be demonstrated using oil red O staining (Neuropathol Appl Neurobiol 1993;19:95)
Frozen section images

Contributed by Valeria Barresi, M.D., Ph.D.
Neurocytic cells

Neurocytic cells

Perivascular fibrillary areas

Perivascular fibrillary areas

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Valeria Barresi, M.D., Ph.D.
Lipidized cells Lipidized cells

Lipidized cells

Perivascular matrix

Perivascular matrix

Synaptophysin immunostaining

Synaptophysin immunostaining

NeuN immunostaining

NeuN immunostaining


GFAP immunostaining

GFAP immunostaining

Olig2 immunostaining

Olig2 immunostaining

EMA immunostaining

EMA immunostaining

Ki67 immunostaining

Ki67 immunostaining

Virtual slides

Images hosted on other servers:
Cerebellar liponeurocytoma

Cerebellar liponeurocytoma

Cytology description
  • Small, uniform cells with rounded to oval nuclei showing salt and pepper chromatin and scant cytoplasm
  • Some cells show a single, large cytoplasmic vacuole displacing the nucleus to the periphery of the cell, similar to adipocytes (Arch Pathol Lab Med 2012;136:965)
Cytology images

Contributed by Valeria Barresi, M.D., Ph.D.
Sheets of uniform neurocytic cells

Sheets of uniform neurocytic cells

Electron microscopy description
Molecular / cytogenetics description
Sample pathology report
  • Cerebellum, tumor resection:
    • Cerebellar liponeurocytoma, CNS WHO grade 2 (see comment)
    • Comment: The tumor is composed of monotonous neurocytic cells, with round nuclei, small nucleoli and scant cytoplasm, disposed in a diffuse growth pattern and showing foci of lipidized cells. Only scattered mitoses are present. Necrosis is absent. On immunohistochemistry, tumor cells are diffusely positive for synaptophysin and MAP2 and negative for Olig2 and EMA. Ki67 labeling index is < 5%.
Differential diagnosis
  • Medulloblastoma:
    • Mainly affects children and young adults
    • Lacks lipidized cells
    • Shows high mitotic index and Ki67 labeling index
    • May show necrosis
    • May occasionally have foamy macrophages that mimic lipomatous differentiation of cerebellar liponeurocytoma
    • Classic subtype is composed of small blue round cells with hyperchromatic nuclei of various shapes
    • Desmoplastic / nodular subtype features reticulin free zones surrounded by densely packed, poorly differentiated, highly proliferative cells with moderately pleomorphic nuclei
    • Large / anaplastic subtype is composed of markedly pleomorphic cells
    • Harbors isochromosome 17q
    • May harbor PTCH, CTNNB and APC mutations
  • Clear cell ependymoma:
    • Shows perivascular pseudorosettes and rosettes
    • Lacks lipidized cells
    • Positive for EMA (dot-like)
    • Diffusely positive for GFAP
    • Negative for synaptophysin
  • Oligodendroglioma, IDH mutant and 1p / 19q codeleted:
    • Rare in the posterior fossa
    • Lacks lipidized cells
    • Positive for Olig2
    • Negative for synaptophysin
    • IDH1 / 2 mutated
    • 1p / 19q codeleted
Board review style question #1

The tumor shown above was found in the left cerebellar hemisphere of a 45 year old woman. Only rare mitoses are present. It shows diffuse immunostaining for synaptophysin, focal immunostaining for GFAP and is negative for Olig2 and EMA. What is the most likely diagnosis?

  1. Cerebellar liponeurocytoma
  2. Ependymoma
  3. Medulloblastoma
  4. Oligodendroglioma
Board review style answer #1
A. Cerebellar liponeurocytoma. This tumor is by definition located in the cerebellum. It is composed of small neurocytic cells with focal lipidized cells, is positive for synaptophysin and has a low mitotic index. Answer C is incorrect because medulloblastoma is not characterized by lipidized cells and it has a high mitotic index. Answer B is incorrect because ependymoma is GFAP positive and features EMA dot-like staining. Answer D is incorrect because oligodendroglioma is diffusely positive for Olig2 and negative for synaptophysin.

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Reference: Cerebellar liponeurocytoma
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