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

Gangliocytoma

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Glioneuronal and neuronal tumours

General

  • Transitional forms between gangliocytoma and ganglioglioma exist: sometimes cannot be separated

Definition

  • Essential:
    • A tumefactive lesion with presence of irregular groups of large, mature ganglion cells AND
    • Matrix resembling normal neuropil, sometimes more coarsely fibrillar or vacuolated
  • Desirable:
    • Atypical and Enucleated ganglion cells cytoplasmic ballooning or vacuolization

Grading

  • WHO Grade 1

Numbers

  • Rare tumours
  • Affecting children

Localisation

  • Throughout CNS
    • Can also occur in pituitary

Clinical features

  • Seizures
    • Like gangliogliomas

Radiological

  • Appearances of gangliocytomas are indistinguishable from gangliogliomas

CT

  • Gangliocytomas typically appears hyperattenuating on non-contrast imaging.
  • Little mass effect
  • Minimal or no surrounding vasogenic oedema → does not enhance much with contrast
  • Calcification and cyst formation can occur.
NCCT
Plain
 
C+ CT
+C
 

MRI

  • T1: solid components typically hypointense
  • T2:
    • Solid components are typically mildly hypointense 2;
    • Cystic areas are hyperintense;
    • Calcification if present can be hypointense
  • T1 C+ (Gd): solid components enhance
Image
T1
T1
A brain scan of a person AI-generated content may be incorrect.
DWI
A close-up of a brain scan AI-generated content may be incorrect.
T1+C
A brain scan of a person AI-generated content may be incorrect.
Gradient Echo
T2 axial
T2 axial
A close-up of a brain scan AI-generated content may be incorrect.
FLAIR

Histopathology

Microscopic

  • Composed of irregular groups of large, multipolar (single axon and many dendrites (and dendritic branches) neurons (often with dysplastic features)
  • Binucleated neurons are commonly observed
  • Density of dysplastic ganglion cells is typically low; it may be close to the density of neurons in grey matter
  • The presence of mitotically active neuroblasts is not compatible with the diagnosis of gangliocytoma and should prompt the differential diagnosis of CNS ganglioneuroblastoma
  • The stroma consists of non-neoplastic glial elements but may be difficult to distinguish from a low-cellularity glial component of a ganglioglioma
Fig. 6.08 Gangliocytoma. A Focal accumulation of multipolar, dysplastic neurons. B Clusters of mature neurons are embedded in an otherwise normal krain matrix. C Stellate ramification of GFAP-positive astrocytes is compatible voth non-neoplastic nature in a gangliocytoma.
Gangliocytoma. A, Focal accumulation of multipolar, dysplastic neurons. B, Clusters of mature neurons are embedded in an otherwise normal brain matrix. C, Stellate ramification of GFAP-positive astrocytes is compatible with non-neoplastic nature in a gangliocytoma.

Immunophenotype

  • +
    • Synaptophysin
    • Neurofilament
    • Chromogranin-A,
    • MAP2
  • -
    • NeuN (weakly expressed or negative)

Genetic

  • None

Prognosis

  • Benign tumours with good outcomes