Neurosurgery notes/Tumours/Gliomas, Glioneuronal Tumors, and Neuronal Tumors/Glioneuronal and neuronal tumours/Ganglioglioma/Anaplastic ganglioglioma

Anaplastic ganglioglioma

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Ganglioglioma

General

  • No longer a recognized entity in the WHO 2021 Classification of CNS Tumors (WHO CNS5)
  • integrating such cases under ganglioglioma or related diagnoses based on integrated histomolecular criteria.
  • The Anaplastic histological features do not represent a poorer outcome

Definition

  • A glioneuronal tumour composed of dysplastic ganglion cells and an anaplastic glial component with elevated mitotic activity.

Number

  • 1– 2.6% of all gangliogliomas

Grading

  • WHO Grade 3

Histopathology

Microscopic

  • Anaplastic changes mainly at glial component
    • Showing:
      • Inc. cellularity
      • Pleomorphism
      • Inc. numbers of mitotic figures
      • Vascular proliferation and necrosis
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Anaplastic ganglioglioma. A, Dysplastic, occasionally binucleated neurons and mitoses within a relatively cellular and pleomorphic astroglial matrix. B, The same tumour contains palisading tumour cells around necrotic foci.
Anaplastic ganglioglioma. A, Dysplastic, occasionally binucleated neurons and mitoses within a relatively cellular and pleomorphic astroglial matrix. B, The same tumour contains palisading tumour cells around necrotic foci.

Genetic profile

  • Losses of CDKN2A/B and DMBT1 or gain/amplification of CDK4 in gangliogliomas are more likely to undergo anaplasia

Treatment

  • Surgery then radiotherapy
    • Not much evidence for it but it is given due to poor prognosis
  • Radiotherapy may be an option in recurrent, unresectable, or incompletely resected tumours with histological signs of atypia, which do not fulfil the criteria for the diagnosis of a WHO grade III tumour.
  • Very few evidence of Chemotherapy

Prognosis

  • 30% 5 yr. PFS: Luyken et al., 2004