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      A case of a rosette-forming glioneuronal tumor arising from the pons with disappearance of contrast enhancement

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          Abstract

          A rosette-forming glioneuronal tumor (RGNT) is a rare and slow-growing central nervous system tumor. This tumor is usually assessed by MRI during the follow-up period. RGNT can show alteration of contrast enhancement regardless of tumor growth. Here, we report a case of RGNT arising from pons which shows partial enhancement on initial MRI, smaller enhancement on follow-up MRI at 10 months, and totally disappeared at 18 months without any therapy.

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          A rosette-forming glioneuronal tumor of the fourth ventricle: infratentorial form of dysembryoplastic neuroepithelial tumor?

          Takanori Hirose, B. Scheithauer, Takashi Komori (2002)
          Eleven cases of a distinctive tumor of the posterior fossa are described. The patients (age range 12-59 years) presented with headache and/or ataxia. Neuroimaging revealed a relatively discrete, focally enhancing mass(es) primarily involving the aqueduct, fourth ventricle, and cerebellar vermis. Hydrocephalus was present in seven cases, and two lesions were multicentric. In two cases a significant increase in tumor size was documented. Gross total or subtotal resections were achieved in 10 cases. One patient underwent biopsy alone and another received postoperative irradiation. Histologically, two components were identified in all cases. One consisted of neurocytes forming neurocytic and/or perivascular pseudorosettes in a fibrillary, partly microcystic matrix. The second, astrocytic component resembled pilocytic astrocytoma in 10 cases and consisted of fibrillated spindle cells with oval nuclei associated with occasional Rosenthal fibers, granular bodies, glomeruloid capillaries, and microcalcifications. Regionally, this component was more diffuse and patternless, consisting of sheets of round to oval, oligodendrocyte-like cells. Rare ganglion cells were seen in four cases. The rosettes were consistently synaptophysin and MAP-2 immunoreactive, whereas the spindle cells were positive for S-100 protein and glial fibrillary acidic protein. Overall, atypia was minimal; no mitoses were found, and Ki67 labeling indices were low. Ultrastructurally, the neurocytic cells featured processes containing microtubules and occasional dense core granules. Mature synapses were found in one of the four cases studied. Although the histologic features of this unique tumor superficially resemble those of dysembryoplastic neuroepithelial tumor, rosette formation by neuronal cells, the frequent presence of a pilocytic astrocytoma component, and the growing nature of the lesion argue against that diagnosis, as does occasional multifocality.
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            Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology.

            Pitt Niehusmann, Hans Clusmann, Paulo R A Campos … (2009)
            Dysembryoplastic neuroepithelial tumors (DNTs) are long-term epilepsy associated tumors subdivided into simple and complex variants. The purpose of this study was to relate different DNT components identified on magnetic resonance imaging (MRI) to histopathological features and to test the hypothesis that glial nodules as a histopathological feature of complex variants induce an occasional glioma misdiagnosis. Clinical, MRI, and histopathologic features of DNTs operated between 1988 and 2008 were reviewed. From a total of 61 DNTs, 48 simple and 13 complex variants were identified. Multiple or single pseudocysts in a cortical/subcortical location with small cysts sometimes separated from the tumor represented the glioneuronal element and were found in all DNTs. FLAIR hyperintense tissue was found between pseudocysts but--in neocortical DNTs--also circumscript in deeper tumor parts. Calcification and hemorrhages in this location occurred in four of 13 complex variants, and one of these patients was also the only one with tumor growth. Patients with complex variants had earlier seizure onset, and complex variants were more often located outside the temporal lobe. Although complex variants represented a higher diagnostic challenge, misdiagnoses also occurred in simple variants. One of five of DNTs showed contrast enhancement, which varied on follow-up studies with enhancing parts becoming nonenhancing and vice versa. The glioneuronal element is readily identifiable on MRI and should be considered to support the DNT diagnosis. Complex DNT variants have a different clinical profile and a more variable histopathological and MRI appearance; however, misdiagnoses occasionally also occur in simple variants.
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              Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system

              CHENLONG YANG, Jingyi Fang, Guang Cun Li … (2017)
              Objective A rosette-forming glioneuronal tumor (RGNT) is a rare entity originally described in the fourth ventricle. Recently, RGNTs occurring in extraventricular sites and those with malignant behaviors have been reported. The purpose of this study was to analyze the clinicoradiological and histopathological features, therapeutic strategies, and outcomes of RGNTs. Methods We enrolled 38 patients diagnosed with RGNTs pathologically between August 2009 and June 2016. CT and MRI, including diffusion-weighted imaging and spectroscopy, were performed. The surgical treatment and histopathological and molecular features were assessed. Additionally, we searched the relevant literatures and performed a pooled analysis of individual patient data. The potential risk factors of prognosis were analyzed. Results Our case series included 22 male and 16 female patients, with a mean age of 25.9 years. RGNTs involved the fourth ventricle (26.3%), cerebella (34.2%), supratentorial ventricular system (13.2%), spinal cord (10.5%), temporal lobe (10.5%), thalamus (7.9%), brain stem (7.9%), frontal lobe (5.3%), pineal region (5.3%), suprasellar region (2.6%), and basal ganglia (2.6%). Statistical analyses showed that pediatric age, purely solid appearance of the tumor, and inadequate resection (only partial removal or biopsy) were risk factors associated with progression events. Patients with subtotal resection appeared to do as well as those with gross total resection. Conclusions RGNTs can occur nearly anywhere in the CNS, at both supratentorial and infratentorial sites. Maximal safe surgical resection should be emphasized for treatment; whilst aggressive resection with the goal of complete resection may be unnecessary.
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                Author and article information

                Contributors
                Masayuki Maeda
                Journal
                Journal ID (nlm-ta): Radiol Case Rep
                Journal ID (iso-abbrev): Radiol Case Rep
                Title: Radiology Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 1930-0433
                Publication date PMC-release: 22 May 2019
                Publication date Collection: August 2019
                Publication date (Electronic): 22 May 2019
                Volume: 14
                Issue: 8
                Pages: 899-902
                Affiliations
                [a ]Department of Radiology, Mie University School of Medicine, Mie, Japan
                [b ]Department of Advanced Diagnostic Imaging, Mie University School of Medicine, Mie, Japan
                [c ]Department of Pathology, Mie University School of Medicine, Mie, Japan
                [d ]Department of Neurosurgery, Mie University School of Medicine, Mie, Japan
                Author notes
                [* ]Corresponding author. mmaeda@ 123456clin.medic.mie-u.ac.jp
                Article
                Publisher ID: S1930-0433(19)30203-1
                DOI: 10.1016/j.radcr.2019.05.011
                PMC ID: 6535692
                PubMed ID: 31193570
                SO-VID: ba2b1fb8-fb79-416b-a21e-bf3999e64118
                Copyright © © 2019 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 20 April 2019
                Date revision received : 9 May 2019
                Date accepted : 9 May 2019
                Categories
                Subject: Neuroradiology

                Keywords: rosette-forming glioneuronal tumor,mri,contrast enhancement
                Data availability:
                Keywords: rosette-forming glioneuronal tumor, mri, contrast enhancement

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