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Rosette-forming glioneuronal tumor

MedGen UID:
1377578
•Concept ID:
C4331262
•
Neoplastic Process
Synonyms: DNT of the cerebellum; dysembryoplastic neuroepithelial tumor of cerebellum; dysembryoplastic neuroepithelial tumour of cerebellum; RGNT; Rosette-forming glioneuronal neoplasm; Rosette-Forming Glioneuronal Tumor; rosette-forming glioneuronal tumor; rosette-forming glioneuronal tumor of fourth ventricule; Rosette-forming glioneuronal tumor of the fourth ventricle; rosette-forming glioneuronal tumour; Rosette-forming glioneuronal tumour of the fourth ventricle
SNOMED CT: Rosette-forming glioneuronal neoplasm (733906003); Rosette-forming glioneuronal tumor (733906003); Rosette-forming glioneuronal neoplasm (770682007); Rosette-forming glioneuronal tumor (770682007)
 
HPO: HP:0025171
Monarch Initiative: MONDO:0016736
Orphanet: ORPHA251975

Definition

A tumor of the central nervous system that has components of both neurocytic and glial areas, whereby usually the glial component of the tumor predominates. Rossette-forming glioneuronal tumors (RGNT) have biphasic cytoarchitecture with two elements; neurocytic rosettes resembling Homer-Wright rosettes, and astrocytic component resembling a pilocytic astrocytoma. RGNTs are low-grade tumors that lack histopathological signs of malignancy. [from HPO]

Professional guidelines

PubMed

Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.
Hockman JT, El Tecle NE, Urquiaga JF, Alexopoulos G, Guzman MA, Coppens J
World Neurosurg 2021 May;149:86-93. Epub 2021 Feb 18 doi: 10.1016/j.wneu.2021.02.043. PMID: 33610864

Recent clinical studies

Etiology

Clinicopathological analysis of rosette-forming glioneuronal tumors.
Liu J, Lin F, Sun Y, Liu X
Diagn Pathol 2024 Feb 22;19(1):39. doi: 10.1186/s13000-024-01465-6. PMID: 38388383Free PMC Article
Immune Microenvironment and Lineage Tracing Help to Decipher Rosette-Forming Glioneuronal Tumors: A Multi-Omics Analysis.
Lerond J, Morisse MC, Letourneur Q, Gimonnet C, Navarro S, Gaspar C, Idbaih A, Bielle F
J Neuropathol Exp Neurol 2022 Oct 18;81(11):873-884. doi: 10.1093/jnen/nlac074. PMID: 35984315
Rosette-Forming Glioneuronal Tumor in the Pineal Region: A Series of 6 Cases and Literature Review.
Lin CC, Mansukhani MM, Bruce JN, Canoll P, Zanazzi G
J Neuropathol Exp Neurol 2021 Oct 26;80(10):933-943. doi: 10.1093/jnen/nlab089. PMID: 34498065
Rosette-forming glioneuronal tumor of the fourth ventricle. Two cases report and literature review.
Choque Cuba B, Ortega Zufiría JM, Poveda Núñez PD, Lomillos Prieto N, Sierra Rodríguez M, Tamarit Degenhardt M, López Serrano R, Gómez Angulo Giner JC, Aramburu González JA
Neurocirugia (Astur : Engl Ed) 2018 Sep-Oct;29(5):255-259. Epub 2018 Jan 12 doi: 10.1016/j.neucir.2017.12.001. PMID: 29338911
Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: report of four cases and literature review.
Marhold F, Preusser M, Dietrich W, Prayer D, Czech T
J Neurooncol 2008 Dec;90(3):301-8. Epub 2008 Sep 7 doi: 10.1007/s11060-008-9661-y. PMID: 18777116

Diagnosis

Clinicopathological analysis of rosette-forming glioneuronal tumors.
Liu J, Lin F, Sun Y, Liu X
Diagn Pathol 2024 Feb 22;19(1):39. doi: 10.1186/s13000-024-01465-6. PMID: 38388383Free PMC Article
Uncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.
Vaz A, Cavalcanti MS, da Silva Junior EB, Ramina R, de Almeida Teixeira BC
AJNR Am J Neuroradiol 2022 Aug;43(8):1080-1089. Epub 2022 May 5 doi: 10.3174/ajnr.A7465. PMID: 35512827Free PMC Article
Rosette-forming glioneuronal tumor: an update.
Anyanwu CT, Robinson TM, Huang JH
Clin Transl Oncol 2020 May;22(5):623-630. Epub 2019 Jul 16 doi: 10.1007/s12094-019-02179-8. PMID: 31313067
MRI of intracranial intraventricular lesions.
Muly S, Liu S, Lee R, Nicolaou S, Rojas R, Khosa F
Clin Imaging 2018 Nov-Dec;52:226-239. Epub 2018 Aug 1 doi: 10.1016/j.clinimag.2018.07.021. PMID: 30138862
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368

Therapy

Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review.
Morris C, Prudowsky ZD, Shetty V, Geller T, Elbabaa SK, Guzman M, AbdelBaki MS
World Neurosurg 2017 Nov;107:1045.e9-1045.e16. Epub 2017 Aug 4 doi: 10.1016/j.wneu.2017.07.150. PMID: 28826709
Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report.
Adachi J, Nishikawa R, Hirose T, Matsutani M
Surg Neurol 2005 Apr;63(4):375-9. doi: 10.1016/j.surneu.2004.05.039. PMID: 15808729

Prognosis

Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.
Hockman JT, El Tecle NE, Urquiaga JF, Alexopoulos G, Guzman MA, Coppens J
World Neurosurg 2021 May;149:86-93. Epub 2021 Feb 18 doi: 10.1016/j.wneu.2021.02.043. PMID: 33610864
Rosette-forming glioneuronal tumor: an update.
Anyanwu CT, Robinson TM, Huang JH
Clin Transl Oncol 2020 May;22(5):623-630. Epub 2019 Jul 16 doi: 10.1007/s12094-019-02179-8. PMID: 31313067
Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review.
Duan L, Zhang Y, Fu W, Geng S
World Neurosurg 2017 Feb;98:875.e1-875.e7. Epub 2016 Nov 30 doi: 10.1016/j.wneu.2016.11.109. PMID: 27915062
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: report of four cases and literature review.
Marhold F, Preusser M, Dietrich W, Prayer D, Czech T
J Neurooncol 2008 Dec;90(3):301-8. Epub 2008 Sep 7 doi: 10.1007/s11060-008-9661-y. PMID: 18777116

Clinical prediction guides

Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.
Hockman JT, El Tecle NE, Urquiaga JF, Alexopoulos G, Guzman MA, Coppens J
World Neurosurg 2021 May;149:86-93. Epub 2021 Feb 18 doi: 10.1016/j.wneu.2021.02.043. PMID: 33610864
Spinal rosette-forming glioneuronal tumor: A case report.
Hamauchi S, Tanino M, Hida K, Sasamori T, Yano S, Tanaka S
Medicine (Baltimore) 2019 Dec;98(49):e18271. doi: 10.1097/MD.0000000000018271. PMID: 31804365Free PMC Article
Comprehensive genetic characterization of rosette-forming glioneuronal tumors: independent component analysis by tissue microdissection.
Kitamura Y, Komori T, Shibuya M, Ohara K, Saito Y, Hayashi S, Sasaki A, Nakagawa E, Tomio R, Kakita A, Nakatsukasa M, Yoshida K, Sasaki H
Brain Pathol 2018 Jan;28(1):87-93. Epub 2017 Feb 9 doi: 10.1111/bpa.12468. PMID: 27893178Free PMC Article
SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors.
Nagaishi M, Nobusawa S, Matsumura N, Kono F, Ishiuchi S, Abe T, Ebato M, Wang Y, Hyodo A, Yokoo H, Nakazato Y
J Clin Neurosci 2016 Jan;23:73-75. Epub 2015 Aug 7 doi: 10.1016/j.jocn.2015.04.021. PMID: 26260115
Glial tumors with neuronal differentiation.
Park CK, Phi JH, Park SH
Neurosurg Clin N Am 2015 Jan;26(1):117-38. doi: 10.1016/j.nec.2014.09.006. PMID: 25432191

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