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      A Rare Lesion in the Anterior Region of the Third Ventricle: Rosette-Forming Glioneuronal Tumor

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          Abstract

          Rosette-forming glioneuronal tumor (RGNT) is a rare and indolent mixed glioneuronal tumor involving primarily the fourth ventricular region and occurring predominantly in young adults.

          We present a case of a 44-year-old woman presented with progressive headaches, vomiting, and a sudden decreasing level of consciousness. The magnetic resonance imaging showed a regular lesion within the anterior portion of the third ventricle and the patient underwent an endoscopic approach to remove the tumor that was exclusively within the anterior portion of the third ventricle. Histopathology showed an RGNT that was totally removed.

          We also report some unusual complications that are described in the literature and are related to ventricular endoscopy such as seizures and hydroelectrolyte disorders.

          With two years of follow-up, the patient had no complaints and no tumor progression was observed.

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          Most cited references20

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          A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord.

          Rosette-forming glioneuronal tumors of the fourth ventricle are rare brain tumors, and only 19 such lesions have been previously reported. This report presents the first case of a rosette-forming glioneuronal tumors arising from the spinal cord. A 44-year-old woman presented with a 15-year history of dissociated sensory disturbance of the lower extremities that gradually spread through her upper extremities. She also experienced continuing motor disturbance. Magnetic resonance imaging demonstrated a mass in the cervicothoracic spinal cord that suggested an intramedullary spinal tumor. A total gross resection of the tumor was performed. As is typical of rosette-forming glioneuronal tumors of the fourth ventricle, this spinal cord example manifested neurocytic and astrocytic components. Neurocytic rosettes were detected in the neurocytic component, and the center of rosettes showed positive immunostaining for synaptophysin. The astrocytic component showed characteristic features of a pilocytic astrocytoma, as is often the case in the fourth ventricle examples.
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            Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination.

            A rosette-forming glioneuronal tumor (RGNT) was encountered in a 16-year-old Chinese girl. She experienced seizures with loss of consciousness for 1 month prior to diagnosis. A brain MRI revealed multifocal masses occupying all of the ventricular system associated with marked hydrocephalus. A biopsy was performed on the right lateral ventricle using a neuroendoscope and the patient was given postoperative radiotherapy. She was followed for 7 months, and there was no radiological or clinical evidence of tumor progression. Histological examination demonstrated two regions characterized by predominant neurocytic rosettes and scant low grade astrocytoma. No histological anaplasia was present. Immunohistological studies showed that the small round tumor cells forming rosettes were strongly positive for Olig2. A chromosome 1p/19q co-deletion was not detected by FISH analysis. While the pathological features of our case closely resembled those reported in the original description, the diffuse intraventricular growth pattern of the tumor was different from previous examples. Further long-term follow-up studies are required to assess the biological behavior and clinical outcome of the RGNT entity.
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              Rosette-forming glioneuronal tumor: report of a chiasmal-optic nerve example in neurofibromatosis type 1: special pathology report.

              Rosette-forming glioneuronal tumor is a rare, rather recently described tumor featuring a highly distinctive, biphasic histological pattern, including a cytologically uniform neuronal component of Homer-Wright type pseudorosettes and an accompanying astrocytic element resembling pilocytic astrocytoma. Its occurrence in the posterior fossa and association with the fourth ventricle is stereotypical and a feature of all reported cases. In this article, we describe the first rosette-forming glioneuronal tumor arising outside this site, a histologically classic example involving the anterior visual pathway and associated with neurofibromatosis type 1. Genetic (fluorescent in situ hybridization) studies demonstrated no large deletion in either normal or neoplastic tissue, indicating that the genetic abnormality underlying neurofibromatosis type 1 in this patient is likely a very small deletion or point mutation. The relation of the tumor to the underlying neurofibromatosis type 1 cannot be assessed.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                9 January 2024
                January 2024
                : 16
                : 1
                : e51971
                Affiliations
                [1 ] Neurosurgery, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, PRT
                [2 ] Neurosurgery, Complexo Hospitalar de Doenças Cardio-Pulmonar Cardial Dom Alexandre do Nascimento, Luanda, AGO
                [3 ] Internal Medicine, Centro Hospitalar de Entre Douro e Vouga, Santa Maria da Feira, PRT
                [4 ] Pathology, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, PRT
                [5 ] Neurological Surgery, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, PRT
                Author notes
                Article
                10.7759/cureus.51971
                10853027
                38333478
                83fe7378-9613-4bd2-9c0a-b9af048448be
                Copyright © 2024, Pereira et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 9 January 2024
                Categories
                Endocrinology/Diabetes/Metabolism
                Neurosurgery

                seizures,endoscopy,cerebral salt-wasting syndrome,third ventricle,rosette-forming glioneuronal tumor

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