• Luke Silveira, John DeWitt, Alissa Thomas, and Bruce Tranmer.
• Department of Neurosurgery, University of Vermont Medical Center, Burlington, Vermont, USA. Electronic address: luke.silveira@uvmhealth.org.
• World Neurosurg. 2019 Dec 1; 132: 7-11.

BackgroundThe rosette-forming glioneuronal tumor (RGNT) is a rare World Health Organization grade I tumor, most often originating in the fourth ventricle and characterized by a predominant glial component, as well as a neurocytic component. Despite its benign grading, there are a few documented cases of RGNT displaying more aggressive behavior with malignant characteristics.Case DescriptionHerein, we describe a uniquely aggressive presentation of RGNT in a 49-year-old man initially presenting with obstructive hydrocephalus with lesions in the right frontal lobe, fourth ventricle, and left lateral ventricle. The patient was found to have 2 distinct intracranial entities: a right frontal meningioma and disseminated intraventricular RGNT. He underwent an anatomically limited resection of the fourth ventricular lesion with fourth ventricle-to-subarachnoid space shunting. His disease progressed thereafter, with diffuse intracranial dissemination and drop metastases in the lumbar spine. He was treated with craniospinal radiation and remains on palliative care 5 months after completion of radiotherapy.ConclusionsThis case supplements and complements the handful of published cases available documenting the evolution of a particularly aggressive case of disseminated RGNT.Copyright © 2019 Elsevier Inc. All rights reserved.

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