Papillary glioneuronal tumour

Case contributed by Dr Bruno Di Muzio

Presentation

First seizure.

Patient Data

Age: 25-year-old

CT Brain

Modality: CT

Ill-defined hyperdense mass within the anterior pole of the left temporal lobe associated with surrounding vasogenic oedema. No midline shift or cerebral herniation. Remainder brain parenchyma is unremarkable. 

MRI Brain

Modality: MRI

The left temporal lobe mass demonstrates a few prominent vessels within associated with a small haemorrhagic foci, has vivid rim enhancement, and shows partial diffusion restriction. Local mass effect and vasogenic oedema are again demonstrated 

Modality: Pathology

MICROSCOPIC DESCRIPTION: 1-3. Sections show a moderately cellular biphasic tumour composed of thin hyalinised blood vessels surrounded by small astrocytes (GFAP+), together with intervening neurocytes (Synaptophysin+) and occasional ganglion cells (NeuN+). There is focal moderate nuclear atypia and occasional mitoses. No pallisaded tumour necrosis or microvascular proliferation with multilayering of atypical cells around vessel lumena. Immunohistochemistry results show tumour cells stain: Nestin Positive (high) NogoA Positive IDH-1 R132H Negative (non-mutated) ATRX Negative (mutated) MGMT Positive (likely non-methylated) p53 Positive p16 CDKN2A Positive EMA Negative CD99 Negative Topoisomerase labelling index: Approximately 25%.

DIAGNOSIS: Brain, left temporal lesion: Papillary glioneuronal tumour (WHO I)

Comment: The histologcal and immunohistochemical features are most consistent with a papillary glioneural tumour (PGNT). A high proliferation index has been previously reported within PGNT, however close clinical follow-up is recommended. Additionally, immunohistochemical results show tumour cells with intact staining for MSH2, MSH6 and MLH1, whilst there is loss of staining for PMS2. Correlation with the known microsatellite instability related bowel lesions (Lynch Syndrome) is recommended. 

Case Discussion

Papillary glioneuronal tumours are a newly recognised entity on the 2007 WHO classification of CNS tumours.They are rare and characterised as a well circumscribed complex solid cystic supratentorial lesion with indolent clinical course.

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Case Information

rID: 47574
Case created: 22nd Aug 2016
Last edited: 29th Mar 2017
Inclusion in quiz mode: Included

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