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Adult embryonal tumor with multilayered rosettes, c19mc-altered

MedGen UID:
83792
•Concept ID:
C0281330
•
Neoplastic Process
Synonyms: Adult Embryonal Tumor with Multilayered Rosettes, C19MC-Altered; adult embryonal tumor with multilayered rosettes, c19mc-altered; adult embryonal tumor with multilayered rosettes, C19MC-altered; adult embryonal tumour with multilayered rosettes, C19MC-altered; Adult Ependymoblastoma; adult ependymoblastoma; ependymoblastoma of adults
 
Monarch Initiative: MONDO:0004310

Definition

An embryonal tumor with multilayered rosettes, C19MC-altered, occurring in adults. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult embryonal tumor with multilayered rosettes, c19mc-altered

Professional guidelines

PubMed

Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children: An International Perspective.
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma.
Franceschi E, Hofer S, Brandes AA, Frappaz D, Kortmann RD, Bromberg J, Dangouloff-Ros V, Boddaert N, Hattingen E, Wiestler B, Clifford SC, Figarella-Branger D, Giangaspero F, Haberler C, Pietsch T, Pajtler KW, Pfister SM, Guzman R, Stummer W, Combs SE, Seidel C, Beier D, McCabe MG, Grotzer M, Laigle-Donadey F, Stücklin ASG, Idbaih A, Preusser M, van den Bent M, Weller M, Hau P
Lancet Oncol 2019 Dec;20(12):e715-e728. doi: 10.1016/S1470-2045(19)30669-2. PMID: 31797797
Advances in Risk Classification and Treatment Strategies for Neuroblastoma.
Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL
J Clin Oncol 2015 Sep 20;33(27):3008-17. Epub 2015 Aug 24 doi: 10.1200/JCO.2014.59.4648. PMID: 26304901Free PMC Article

Recent clinical studies

Etiology

How we treat medulloblastoma in adults.
Franceschi E, Seidel C, Sahm F, Pajtler KW, Hau P
ESMO Open 2021 Aug;6(4):100173. Epub 2021 Jun 9 doi: 10.1016/j.esmoop.2021.100173. PMID: 34118771Free PMC Article
Children's Oncology Group Phase III Trial of Reduced-Dose and Reduced-Volume Radiotherapy With Chemotherapy for Newly Diagnosed Average-Risk Medulloblastoma.
Michalski JM, Janss AJ, Vezina LG, Smith KS, Billups CA, Burger PC, Embry LM, Cullen PL, Hardy KK, Pomeroy SL, Bass JK, Perkins SM, Merchant TE, Colte PD, Fitzgerald TJ, Booth TN, Cherlow JM, Muraszko KM, Hadley J, Kumar R, Han Y, Tarbell NJ, Fouladi M, Pollack IF, Packer RJ, Li Y, Gajjar A, Northcott PA
J Clin Oncol 2021 Aug 20;39(24):2685-2697. Epub 2021 Jun 10 doi: 10.1200/JCO.20.02730. PMID: 34110925Free PMC Article
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313
Neuroblastoma.
Shah S, Ravindranath Y
Indian J Pediatr 1998 Sep-Oct;65(5):691-705. doi: 10.1007/BF02731044. PMID: 10773924
Esthesioneuroblastoma.
Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

Diagnosis

Intraocular Medulloepitheliomas and Embryonal Tumors With Multilayered Rosettes of the Brain: Comparative Roles of LIN28A and C19MC.
Jakobiec FA, Kool M, Stagner AM, Pfister SM, Eagle RC, Proia AD, Korshunov A
Am J Ophthalmol 2015 Jun;159(6):1065-1074.e1. Epub 2015 Mar 5 doi: 10.1016/j.ajo.2015.03.002. PMID: 25748578
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687
Medulloepithelioma (diktyoma).
Canning CR, McCartney AC, Hungerford J
Br J Ophthalmol 1988 Oct;72(10):764-7. doi: 10.1136/bjo.72.10.764. PMID: 3056510Free PMC Article
Esthesioneuroblastoma.
Shah JP, Feghali J
Am J Surg 1981 Oct;142(4):456-8. doi: 10.1016/0002-9610(81)90374-3. PMID: 7283046
Esthesioneuroblastoma.
Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

Therapy

Immunotherapy of Neuroblastoma: Facts and Hopes.
Anderson J, Majzner RG, Sondel PM
Clin Cancer Res 2022 Aug 2;28(15):3196-3206. doi: 10.1158/1078-0432.CCR-21-1356. PMID: 35435953Free PMC Article
Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group.
Leary SES, Packer RJ, Li Y, Billups CA, Smith KS, Jaju A, Heier L, Burger P, Walsh K, Han Y, Embry L, Hadley J, Kumar R, Michalski J, Hwang E, Gajjar A, Pollack IF, Fouladi M, Northcott PA, Olson JM
JAMA Oncol 2021 Sep 1;7(9):1313-1321. doi: 10.1001/jamaoncol.2021.2224. PMID: 34292305Free PMC Article
The immune landscape of neuroblastoma: Challenges and opportunities for novel therapeutic strategies in pediatric oncology.
Wienke J, Dierselhuis MP, Tytgat GAM, Künkele A, Nierkens S, Molenaar JJ
Eur J Cancer 2021 Feb;144:123-150. Epub 2020 Dec 18 doi: 10.1016/j.ejca.2020.11.014. PMID: 33341446
Advances in Risk Classification and Treatment Strategies for Neuroblastoma.
Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL
J Clin Oncol 2015 Sep 20;33(27):3008-17. Epub 2015 Aug 24 doi: 10.1200/JCO.2014.59.4648. PMID: 26304901Free PMC Article
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313

Prognosis

Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.
Wright A, Desai M, Bolan CW, Badawy M, Guccione J, Rao Korivi B, Pickhardt PJ, Mellnick VM, Lubner MG, Chen L, Elsayes KM
Radiographics 2022 Jul-Aug;42(4):1145-1160. Epub 2022 May 27 doi: 10.1148/rg.210226. PMID: 35622491
Pediatric vulvar malignancies: rare but important to know.
Strickland AL, Fadare O
Semin Diagn Pathol 2021 Jan;38(1):99-109. Epub 2020 Sep 5 doi: 10.1053/j.semdp.2020.09.001. PMID: 32943238
Intraocular Medulloepitheliomas and Embryonal Tumors With Multilayered Rosettes of the Brain: Comparative Roles of LIN28A and C19MC.
Jakobiec FA, Kool M, Stagner AM, Pfister SM, Eagle RC, Proia AD, Korshunov A
Am J Ophthalmol 2015 Jun;159(6):1065-1074.e1. Epub 2015 Mar 5 doi: 10.1016/j.ajo.2015.03.002. PMID: 25748578
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS
N Engl J Med 2003 Feb 20;348(8):694-701. doi: 10.1056/NEJMoa020890. PMID: 12594313
Esthesioneuroblastoma.
Elkon D, Hightower SI, Lim ML, Cantrell RW, Constable WC
Cancer 1979 Sep;44(3):1087-94. doi: 10.1002/1097-0142(197909)44:3<1087::aid-cncr2820440343>3.0.co;2-a. PMID: 383268

Clinical prediction guides

FDA Approval Summary: Belzutifan for von Hippel-Lindau Disease-Associated Tumors.
Fallah J, Brave MH, Weinstock C, Mehta GU, Bradford D, Gittleman H, Bloomquist EW, Charlab R, Hamed SS, Miller CP, Dorff SE, Chambers WA, Mixter BD, Dinin J, Pierce WF, Ricks TK, Tang S, Donoghue M, Pazdur R, Amiri-Kordestani L, Ibrahim A, Beaver JA
Clin Cancer Res 2022 Nov 14;28(22):4843-4848. doi: 10.1158/1078-0432.CCR-22-1054. PMID: 35727604Free PMC Article
The immune cell atlas of human neuroblastoma.
Verhoeven BM, Mei S, Olsen TK, Gustafsson K, Valind A, Lindström A, Gisselsson D, Fard SS, Hagerling C, Kharchenko PV, Kogner P, Johnsen JI, Baryawno N
Cell Rep Med 2022 Jun 21;3(6):100657. Epub 2022 Jun 9 doi: 10.1016/j.xcrm.2022.100657. PMID: 35688160Free PMC Article
Outcomes by Clinical and Molecular Features in Children With Medulloblastoma Treated With Risk-Adapted Therapy: Results of an International Phase III Trial (SJMB03).
Gajjar A, Robinson GW, Smith KS, Lin T, Merchant TE, Chintagumpala M, Mahajan A, Su J, Bouffet E, Bartels U, Schechter T, Hassall T, Robertson T, Nicholls W, Gururangan S, Schroeder K, Sullivan M, Wheeler G, Hansford JR, Kellie SJ, McCowage G, Cohn R, Fisher MJ, Krasin MJ, Stewart CF, Broniscer A, Buchhalter I, Tatevossian RG, Orr BA, Neale G, Klimo P Jr, Boop F, Srinivasan A, Pfister SM, Gilbertson RJ, Onar-Thomas A, Ellison DW, Northcott PA
J Clin Oncol 2021 Mar 1;39(7):822-835. Epub 2021 Jan 6 doi: 10.1200/JCO.20.01372. PMID: 33405951Free PMC Article
Spectrum and prevalence of genetic predisposition in medulloblastoma: a retrospective genetic study and prospective validation in a clinical trial cohort.
Waszak SM, Northcott PA, Buchhalter I, Robinson GW, Sutter C, Groebner S, Grund KB, Brugières L, Jones DTW, Pajtler KW, Morrissy AS, Kool M, Sturm D, Chavez L, Ernst A, Brabetz S, Hain M, Zichner T, Segura-Wang M, Weischenfeldt J, Rausch T, Mardin BR, Zhou X, Baciu C, Lawerenz C, Chan JA, Varlet P, Guerrini-Rousseau L, Fults DW, Grajkowska W, Hauser P, Jabado N, Ra YS, Zitterbart K, Shringarpure SS, De La Vega FM, Bustamante CD, Ng HK, Perry A, MacDonald TJ, Hernáiz Driever P, Bendel AE, Bowers DC, McCowage G, Chintagumpala MM, Cohn R, Hassall T, Fleischhack G, Eggen T, Wesenberg F, Feychting M, Lannering B, Schüz J, Johansen C, Andersen TV, Röösli M, Kuehni CE, Grotzer M, Kjaerheim K, Monoranu CM, Archer TC, Duke E, Pomeroy SL, Shelagh R, Frank S, Sumerauer D, Scheurlen W, Ryzhova MV, Milde T, Kratz CP, Samuel D, Zhang J, Solomon DA, Marra M, Eils R, Bartram CR, von Hoff K, Rutkowski S, Ramaswamy V, Gilbertson RJ, Korshunov A, Taylor MD, Lichter P, Malkin D, Gajjar A, Korbel JO, Pfister SM
Lancet Oncol 2018 Jun;19(6):785-798. Epub 2018 May 9 doi: 10.1016/S1470-2045(18)30242-0. PMID: 29753700Free PMC Article
Advances in Risk Classification and Treatment Strategies for Neuroblastoma.
Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL
J Clin Oncol 2015 Sep 20;33(27):3008-17. Epub 2015 Aug 24 doi: 10.1200/JCO.2014.59.4648. PMID: 26304901Free PMC Article

Recent systematic reviews

The SH-SY5Y human neuroblastoma cell line, a relevant in vitro cell model for investigating neurotoxicology in human: Focus on organic pollutants.
Lopez-Suarez L, Awabdh SA, Coumoul X, Chauvet C
Neurotoxicology 2022 Sep;92:131-155. Epub 2022 Jul 29 doi: 10.1016/j.neuro.2022.07.008. PMID: 35914637
Patient-Derived Xenotransplant of CNS Neoplasms in Zebrafish: A Systematic Review.
Sarmiento BE, Callegari S, Ghotme KA, Akle V
Cells 2022 Apr 2;11(7) doi: 10.3390/cells11071204. PMID: 35406768Free PMC Article
Management of recurrent medulloblastoma in adult patients: a systematic review and recommendations.
Kostaras X, Easaw JC
J Neurooncol 2013 Oct;115(1):1-8. Epub 2013 Jul 23 doi: 10.1007/s11060-013-1206-3. PMID: 23877361
Endoscopic endonasal compared with anterior craniofacial and combined cranionasal resection of esthesioneuroblastomas.
Komotar RJ, Starke RM, Raper DM, Anand VK, Schwartz TH
World Neurosurg 2013 Jul-Aug;80(1-2):148-59. Epub 2012 Dec 7 doi: 10.1016/j.wneu.2012.12.003. PMID: 23228365
Breast-feeding and childhood cancer: A systematic review with metaanalysis.
Martin RM, Gunnell D, Owen CG, Smith GD
Int J Cancer 2005 Dec 20;117(6):1020-31. doi: 10.1002/ijc.21274. PMID: 15986434

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