Diffuse leptomeningeal glioneuronal tumor

Case contributed by Jeremy Jones
Diagnosis certain


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

Patient Data

Age: 14 years
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 transudation.

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 artifact is demonstrated at the foramen of Monro, cerebral aqueduct and foramina of Luschka. 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 tumor (also known as disseminated oligodendroglial-like leptomeningeal tumor of childhood), a relatively recently described tumor only added to the WHO classification of CNS tumors in the 2016 update. 

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