Diffuse leptomeningeal glioneuronal tumour

Case contributed by Dr Jeremy Jones

Presentation

Generalised headaches for several months. Increased headaches recently. Vomiting for 2 days. Symptoms worsening. No rise in inflammatory markers.

Patient Data

Age: 14
Gender: Female

Widespread meningeal enhancement, particularly around the brainstem with nodularity. Nodular enhancement extends to the ventricular surface.

The lateral ventricles, third ventricle and fourth ventricle are all dilated with transependymal CSF spread.

Features here are of a diffuse process causing hydrocephalus at the level of the fourth ventricle.

Widespread meningeal enhancement over the surface of the cord, particularly in the cervicothoracic region. No focal cord lesion.

Case Discussion

Ventricular dilatation and CSF transudation are present and CSF flow artefact is demonstrated at the foramen of Monro, cerebral aqueduct and foramina of Magendie. Features here are of communicating hydrocephalus.

There is widespread leptomeningeal enhancement. There is nodular leptomeningeal disease that extends around the upper cervical cord.

There is also nodular ependymal enhancement within the lateral ventricles.

Biopsy of abnormal meningeal tissues from around the base of the brain was performed and revealed a highly cellular malignant process. The final report confirmed this to be diffuse leptomeningeal glioneuronal tumour (also known as disseminated oligodendroglial-like leptomeningeal tumour of childhood) a relatively recently described tumour only added to the WHO classification of CNS tumours in the 2016 update. 

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Case information

rID: 32561
Case created: 2nd Dec 2014
Last edited: 13th Jun 2016
Inclusion in quiz mode: Included

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