Papillary glioneuronal tumour

Dr Amir Rezaee et al.

Papillary glioneuronal tumours are rare well circumscribed complex solid cystic supratentorial lesion with an indolent clinical course.

These tumours typically are diagnosed in younger patients (median age at diagnosis 23 years) but are reported essentially in all ages 4. No sex predilection has been identified 4

Clinical presentation is non-specific, primarily comprising seizures and/or headaches. Focal neurological deficits may also be present. 

Papillary glioneuronal tumours were first recognised in the 2007 edition of the WHO classification of CNS tumours and are considered WHO grade I tumours.

The majority of these tumours are in the cerebral hemispheres, with occasional intraventricular tumours described 4

Macroscopic appearance

These tumours usually demonstrate a mixture of solid and cystic components, sometimes with calcification 4

Microscopic appearance

Papillary glioneuronal tumours contain both glial (astrocytic) and neural elements. Characteristic microscopic features are hyalinized vascular pseudopapillary architecture with a pseudostratified layer of cuboidal glial cells and focal collections of neurocytes and ganglion cells.

Immunophenotype

Immunohistochemistry of the cuboidal glial cells reflects their glial appearance 4

In contrast, the neuronal components show a separate immunophenotype 4:

These tumours demonstrate a low Ki-67 proliferation index/MIB-1 index ~1-2% 1-4. Rarely, tumours with higher mitotic index are encountered and some have more aggressive behaviour 4

Features on imaging are very similar to ganglioglioma. They are supratentorial solid-cystic or cystic with solid nodule lesions showing intense but heterogeneous enhancement. Calcification is a frequent finding 1-4. Haemorrhage is seen in ~10% of cases and is sometimes very pronounced 4

MRI
  • T1: isointense to hypointense 
  • T2: inhomogeneously hyperintense
  • FLAIR:
    • inhomogeneously hyperintense nodule
    • cyst may suppress
  • T1 C+: avid but heterogeneous enhancement of the solid nodule 

Papillary glioneuronal tumours are indolent and surgical resection is usually sufficient to effect a cure. In cases where proliferation is high (e.g. Ki-67 >5%) then local recurrence has been described 4.  

The differential diagnosis is primarily of other parenchymal tumours with mixed solid and cystic components, including:

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Article Information

rID: 35516
Section: Pathology
Synonyms or Alternate Spellings:
  • Papillary glioneuronal tumours
  • Papillary glioneuronal tumor
  • Papillary glioneuronal tumors
  • Pseudopapillary ganglioglioneurocytoma
  • Pseudopapillary neurocytoma with glial differentiation

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