U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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 in Opticochiasmatic Region-Novel Entity in New Location.
Sekar A, Rudrappa S, Gopal S, Ghosal N, Rai A
World Neurosurg 2019 May;125:253-256. Epub 2019 Feb 18 doi: 10.1016/j.wneu.2019.02.003. PMID: 30790744
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

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
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
Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update.
Beuriat PA, Tauziede-Espariat A, Pages M, Varlet P, Di Rocco F
Childs Nerv Syst 2016 Jan;32(1):65-8. Epub 2015 Oct 5 doi: 10.1007/s00381-015-2922-0. PMID: 26438552

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

The rosette-forming glioneuronal tumor mimicked cerebral cysticercosis: a case report.
Zhu D, Cheng A, Benons NTL, Chu S
Neurol Sci 2021 Oct;42(10):4301-4308. Epub 2021 May 20 doi: 10.1007/s10072-021-05199-x. PMID: 34018072Free PMC Article
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 in Opticochiasmatic Region-Novel Entity in New Location.
Sekar A, Rudrappa S, Gopal S, Ghosal N, Rai A
World Neurosurg 2019 May;125:253-256. Epub 2019 Feb 18 doi: 10.1016/j.wneu.2019.02.003. PMID: 30790744
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

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
Malignant Transformation of a Rosette-Forming Glioneuronal Tumor to Glioblastoma.
Kwon SM, Kim JH, Byun J, Kim YH, Hong SH, Cho YH, Kim CJ
World Neurosurg 2019 Oct;130:271-275. Epub 2019 Jun 14 doi: 10.1016/j.wneu.2019.06.042. PMID: 31203071
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...