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Embryonal Tumor with Multilayered Rosettes without C19MC Alteration

MedGen UID:
1388361
•Concept ID:
C4329787
•
Neoplastic Process
Synonyms: Embryonal Tumor with Multilayered Rosettes, NOS; Embryonal Tumor with Multilayered Rosettes, Not Otherwise Specified

Definition

An embryonal tumor with multilayered rosettes characterized by the absence of C19MC amplification. Approximately half of embryonal tumors with multilayered rosettes that lack a C19MC alteration carry DICER1 mutations. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEmbryonal Tumor with Multilayered Rosettes without C19MC Alteration

Professional guidelines

PubMed

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179

Recent clinical studies

Etiology

Classification of Brain Tumors by Nanopore Sequencing of Cell-Free DNA from Cerebrospinal Fluid.
Afflerbach AK, Rohrandt C, Brändl B, Sönksen M, Hench J, Frank S, Börnigen D, Alawi M, Mynarek M, Winkler B, Ricklefs F, Synowitz M, Dührsen L, Rutkowski S, Wefers AK, Müller FJ, Schoof M, Schüller U
Clin Chem 2024 Jan 4;70(1):250-260. doi: 10.1093/clinchem/hvad115. PMID: 37624932
Embryonal tumors in the WHO CNS5 classification: A Review.
Gianno F, Miele E, Antonelli M, Giangaspero F
Indian J Pathol Microbiol 2022 May;65(Supplement):S73-S82. doi: 10.4103/ijpm.ijpm_1049_21. PMID: 35562137
C19MC amplification and expression of Lin28A and Olig2 in the classification of embryonal tumors of the central nervous system: A 14-year retrospective study from a tertiary care center.
Nambirajan A, Gurung N, Suri V, Sarkar C, Kumar A, Singh M, Sharma MC
Childs Nerv Syst 2021 Apr;37(4):1067-1075. Epub 2020 Nov 24 doi: 10.1007/s00381-020-04973-0. PMID: 33236184
Embryonal Tumor with Multilayered Rosettes, C19MC-Altered: Clinical, Pathological, and Neuroimaging Findings.
Wang B, Gogia B, Fuller GN, Ketonen LM
J Neuroimaging 2018 Sep;28(5):483-489. Epub 2018 May 24 doi: 10.1111/jon.12524. PMID: 29797626
CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity.
Spence T, Sin-Chan P, Picard D, Barszczyk M, Hoss K, Lu M, Kim SK, Ra YS, Nakamura H, Fangusaro J, Hwang E, Kiehna E, Toledano H, Wang Y, Shi Q, Johnston D, Michaud J, La Spina M, Buccoliero AM, Adamek D, Camelo-Piragua S, Peter Collins V, Jones C, Kabbara N, Jurdi N, Varlet P, Perry A, Scharnhorst D, Fan X, Muraszko KM, Eberhart CG, Ng HK, Gururangan S, Van Meter T, Remke M, Lafay-Cousin L, Chan JA, Sirachainan N, Pomeroy SL, Clifford SC, Gajjar A, Shago M, Halliday W, Taylor MD, Grundy R, Lau CC, Phillips J, Bouffet E, Dirks PB, Hawkins CE, Huang A
Acta Neuropathol 2014 Aug;128(2):291-303. Epub 2014 May 20 doi: 10.1007/s00401-014-1291-1. PMID: 24839957Free PMC Article

Diagnosis

Classification of Brain Tumors by Nanopore Sequencing of Cell-Free DNA from Cerebrospinal Fluid.
Afflerbach AK, Rohrandt C, Brändl B, Sönksen M, Hench J, Frank S, Börnigen D, Alawi M, Mynarek M, Winkler B, Ricklefs F, Synowitz M, Dührsen L, Rutkowski S, Wefers AK, Müller FJ, Schoof M, Schüller U
Clin Chem 2024 Jan 4;70(1):250-260. doi: 10.1093/clinchem/hvad115. PMID: 37624932
Embryonal tumors in the WHO CNS5 classification: A Review.
Gianno F, Miele E, Antonelli M, Giangaspero F
Indian J Pathol Microbiol 2022 May;65(Supplement):S73-S82. doi: 10.4103/ijpm.ijpm_1049_21. PMID: 35562137
ETMR: a tumor entity in its infancy.
Lambo S, von Hoff K, Korshunov A, Pfister SM, Kool M
Acta Neuropathol 2020 Sep;140(3):249-266. Epub 2020 Jun 29 doi: 10.1007/s00401-020-02182-2. PMID: 32601913Free PMC Article
The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW
Acta Neuropathol 2016 Jun;131(6):803-20. Epub 2016 May 9 doi: 10.1007/s00401-016-1545-1. PMID: 27157931
CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity.
Spence T, Sin-Chan P, Picard D, Barszczyk M, Hoss K, Lu M, Kim SK, Ra YS, Nakamura H, Fangusaro J, Hwang E, Kiehna E, Toledano H, Wang Y, Shi Q, Johnston D, Michaud J, La Spina M, Buccoliero AM, Adamek D, Camelo-Piragua S, Peter Collins V, Jones C, Kabbara N, Jurdi N, Varlet P, Perry A, Scharnhorst D, Fan X, Muraszko KM, Eberhart CG, Ng HK, Gururangan S, Van Meter T, Remke M, Lafay-Cousin L, Chan JA, Sirachainan N, Pomeroy SL, Clifford SC, Gajjar A, Shago M, Halliday W, Taylor MD, Grundy R, Lau CC, Phillips J, Bouffet E, Dirks PB, Hawkins CE, Huang A
Acta Neuropathol 2014 Aug;128(2):291-303. Epub 2014 May 20 doi: 10.1007/s00401-014-1291-1. PMID: 24839957Free PMC Article

Therapy

Classification of Brain Tumors by Nanopore Sequencing of Cell-Free DNA from Cerebrospinal Fluid.
Afflerbach AK, Rohrandt C, Brändl B, Sönksen M, Hench J, Frank S, Börnigen D, Alawi M, Mynarek M, Winkler B, Ricklefs F, Synowitz M, Dührsen L, Rutkowski S, Wefers AK, Müller FJ, Schoof M, Schüller U
Clin Chem 2024 Jan 4;70(1):250-260. doi: 10.1093/clinchem/hvad115. PMID: 37624932
Embryonal Tumors of the Central Nervous System: An Update.
Blessing MM, Alexandrescu S
Surg Pathol Clin 2020 Jun;13(2):235-247. Epub 2020 Apr 7 doi: 10.1016/j.path.2020.01.003. PMID: 32389264
Long-term survival in a case of ETANTR with histological features of neuronal maturation after therapy.
Antonelli M, Korshunov A, Mastronuzzi A, Diomedi Camassei F, Carai A, Colafati GS, Pfister SM, Kool M, Giangaspero F
Virchows Arch 2015 May;466(5):603-7. Epub 2015 Feb 20 doi: 10.1007/s00428-015-1736-5. PMID: 25697539

Prognosis

Classification of Brain Tumors by Nanopore Sequencing of Cell-Free DNA from Cerebrospinal Fluid.
Afflerbach AK, Rohrandt C, Brändl B, Sönksen M, Hench J, Frank S, Börnigen D, Alawi M, Mynarek M, Winkler B, Ricklefs F, Synowitz M, Dührsen L, Rutkowski S, Wefers AK, Müller FJ, Schoof M, Schüller U
Clin Chem 2024 Jan 4;70(1):250-260. doi: 10.1093/clinchem/hvad115. PMID: 37624932
Embryonal tumors in the WHO CNS5 classification: A Review.
Gianno F, Miele E, Antonelli M, Giangaspero F
Indian J Pathol Microbiol 2022 May;65(Supplement):S73-S82. doi: 10.4103/ijpm.ijpm_1049_21. PMID: 35562137
Clinical phenotypes and prognostic features of embryonal tumours with multi-layered rosettes: a Rare Brain Tumor Registry study.
Khan S, Solano-Paez P, Suwal T, Lu M, Al-Karmi S, Ho B, Mumal I, Shago M, Hoffman LM, Dodgshun A, Nobusawa S, Tabori U, Bartels U, Ziegler DS, Hansford JR, Ramaswamy V, Hawkins C, Dufour C, André N, Bouffet E, Huang A; Rare Brain Tumor Registry
Lancet Child Adolesc Health 2021 Nov;5(11):800-813. Epub 2021 Sep 30 doi: 10.1016/S2352-4642(21)00245-5. PMID: 34599879
Embryonal Tumors of the Central Nervous System: An Update.
Blessing MM, Alexandrescu S
Surg Pathol Clin 2020 Jun;13(2):235-247. Epub 2020 Apr 7 doi: 10.1016/j.path.2020.01.003. PMID: 32389264
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179

Clinical prediction guides

Tissue Factor Regulation by miR-520g in Primitive Neuronal Brain Tumor Cells: A Possible Link between Oncomirs and the Vascular Tumor Microenvironment.
D'Asti E, Huang A, Kool M, Meehan B, Chan JA, Jabado N, Korshunov A, Pfister SM, Rak J
Am J Pathol 2016 Feb;186(2):446-59. Epub 2015 Dec 12 doi: 10.1016/j.ajpath.2015.10.020. PMID: 26687818Free PMC Article
Long-term survival in a case of ETANTR with histological features of neuronal maturation after therapy.
Antonelli M, Korshunov A, Mastronuzzi A, Diomedi Camassei F, Carai A, Colafati GS, Pfister SM, Kool M, Giangaspero F
Virchows Arch 2015 May;466(5):603-7. Epub 2015 Feb 20 doi: 10.1007/s00428-015-1736-5. PMID: 25697539

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