Luke Silveira1, John DeWitt2, Alissa Thomas3, Bruce Tranmer4. 1. Department of Neurosurgery, University of Vermont Medical Center, Burlington, Vermont, USA. Electronic address: luke.silveira@uvmhealth.org. 2. Department of Neuropathology, University of Vermont Medical Center, Burlington, Vermont, USA. 3. Department of Neurology, University of Vermont Medical Center, Burlington, Vermont, USA. 4. Department of Neurosurgery, University of Vermont Medical Center, Burlington, Vermont, USA.
Abstract
BACKGROUND: The 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 DESCRIPTION: Herein, 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. CONCLUSIONS: This case supplements and complements the handful of published cases available documenting the evolution of a particularly aggressive case of disseminated RGNT.
BACKGROUND: The 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 DESCRIPTION: Herein, 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. CONCLUSIONS: This case supplements and complements the handful of published cases available documenting the evolution of a particularly aggressive case of disseminated RGNT.
Authors: Caleb P Wilson; Arpan R Chakraborty; Panayiotis E Pelargos; Helen H Shi; Camille K Milton; Sarah Sung; Tressie McCoy; Jo Elle Peterson; Chad A Glenn Journal: Neurooncol Adv Date: 2020-09-09