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Childhood supratentorial embryonal tumor with multilayered rosettes, C19MC-altered

MedGen UID:
1375924
•Concept ID:
C4316809
•
Neoplastic Process
Synonyms: Childhood Supratentorial Embryonal Tumor with Multilayered Rosettes, C19MC-Altered; childhood supratentorial embryonal tumor with multilayered rosettes, C19MC-altered; Childhood Supratentorial Ependymoblastoma; Pediatric Supratentorial Ependymoblastoma
 
Monarch Initiative: MONDO:0850388

Definition

An embryonal tumor with multilayered rosettes, C19MC-altered that arises from the supratentorial brain and occurs in children. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood supratentorial embryonal tumor with multilayered rosettes, C19MC-altered

Professional guidelines

PubMed

Pathology, treatment and management of posterior fossa brain tumors in childhood.
Bonner K, Siegel KR
J Neurosci Nurs 1988 Apr;20(2):84-93. doi: 10.1097/01376517-198804000-00004. PMID: 2966218
Treatment of brain tumors in babies and very young children.
Duffner PK, Cohen ME
Pediatr Neurosci 1985-1986;12(6):304-10. doi: 10.1159/000120271. PMID: 3916645
Diagnosis and surgical treatment of childhood brain tumors.
Wilson CB
Cancer 1975 Mar;35(3 suppl):950-6. doi: 10.1002/1097-0142(197503)35:3+<950::aid-cncr2820350716>3.0.co;2-l. PMID: 1167486

Recent clinical studies

Etiology

Maternal Prenatal Use of Alcohol, Tobacco, and Illicit Drugs and Associations with Childhood Cancer Subtypes.
Wimberly CE, Gulrajani NB, Russ JB, Landi D, Wiemels JL, Towry L, Wiencke JK, Walsh KM
Cancer Epidemiol Biomarkers Prev 2024 Mar 1;33(3):347-354. doi: 10.1158/1055-9965.EPI-23-1027. PMID: 38112788Free PMC Article
Supratentorial CNS-PNETs in children; a Swedish population-based study with molecular re-evaluation and long-term follow-up.
Schepke E, Löfgren M, Pietsch T, Kling T, Nordborg C, Olsson Bontell T, Holm S, Öberg A, Nyman P, Eliasson-Hofvander M, Sabel M, Lannering B, Carén H
Clin Epigenetics 2023 Mar 9;15(1):40. doi: 10.1186/s13148-023-01456-2. PMID: 36895035Free PMC Article
Oral and dental late effects in long-term survivors of childhood embryonal brain tumors.
Tanem KE, Stensvold E, Wilberg P, Skaare AB, Brandal P, Herlofson BB
Support Care Cancer 2022 Dec;30(12):10233-10241. Epub 2022 Oct 29 doi: 10.1007/s00520-022-07405-8. PMID: 36307656Free PMC Article
Significance and therapeutic value of miRNAs in embryonal neural tumors.
Shalaby T, Fiaschetti G, Baumgartner M, Grotzer MA
Molecules 2014 May 6;19(5):5821-62. doi: 10.3390/molecules19055821. PMID: 24806581Free PMC Article
Recent advances in paediatric neuro-oncology.
Saran F
Curr Opin Neurol 2002 Dec;15(6):671-7. doi: 10.1097/01.wco.0000044762.39452.e3. PMID: 12447104

Diagnosis

Supratentorial CNS-PNETs in children; a Swedish population-based study with molecular re-evaluation and long-term follow-up.
Schepke E, Löfgren M, Pietsch T, Kling T, Nordborg C, Olsson Bontell T, Holm S, Öberg A, Nyman P, Eliasson-Hofvander M, Sabel M, Lannering B, Carén H
Clin Epigenetics 2023 Mar 9;15(1):40. doi: 10.1186/s13148-023-01456-2. PMID: 36895035Free PMC Article
Bone mineral density and nutrition in long-term survivors of childhood brain tumors.
Kvammen JA, Stensvold E, Godang K, Bollerslev J, Myklebust TÅ, Brandal P, Henriksen C, Bechensteen AG
Clin Nutr ESPEN 2022 Aug;50:162-169. Epub 2022 Jun 4 doi: 10.1016/j.clnesp.2022.05.025. PMID: 35871919
Taste and smell function in long-term survivors after childhood medulloblastoma/CNS-PNET.
Tanem KE, Stensvold E, Wilberg P, Skaare AB, Singh PB, Brandal P, Herlofson BB
Support Care Cancer 2022 Jul;30(7):6155-6162. Epub 2022 Apr 15 doi: 10.1007/s00520-022-07048-9. PMID: 35426047Free PMC Article
Significance and therapeutic value of miRNAs in embryonal neural tumors.
Shalaby T, Fiaschetti G, Baumgartner M, Grotzer MA
Molecules 2014 May 6;19(5):5821-62. doi: 10.3390/molecules19055821. PMID: 24806581Free PMC Article
Temporal trends among childhood brain tumor biopsies. The Childhood Brain Tumor Consortium.
Gilles FH, Sobel EL, Leviton A, Hedley-Whyte ET, Tavare CJ, Adelman L, Sobel RA
J Neurooncol 1992 Jun;13(2):137-49. doi: 10.1007/BF00172763. PMID: 1432031

Therapy

Brain and Brain Stem Necrosis After Reirradiation for Recurrent Childhood Primary Central Nervous System Tumors: A PENTEC Comprehensive Review.
Ajithkumar T, Avanzo M, Yorke E, Tsang DS, Milano MT, Olch AJ, Merchant TE, Dieckmann K, Mahajan A, Fuji H, Paulino AC, Timmermann B, Marks LB, Bentzen SM, Jackson A, Constine LS
Int J Radiat Oncol Biol Phys 2024 Jun 1;119(2):655-668. Epub 2024 Feb 1 doi: 10.1016/j.ijrobp.2023.12.043. PMID: 38300187
Maternal Prenatal Use of Alcohol, Tobacco, and Illicit Drugs and Associations with Childhood Cancer Subtypes.
Wimberly CE, Gulrajani NB, Russ JB, Landi D, Wiemels JL, Towry L, Wiencke JK, Walsh KM
Cancer Epidemiol Biomarkers Prev 2024 Mar 1;33(3):347-354. doi: 10.1158/1055-9965.EPI-23-1027. PMID: 38112788Free PMC Article
Recent advances in paediatric neuro-oncology.
Saran F
Curr Opin Neurol 2002 Dec;15(6):671-7. doi: 10.1097/01.wco.0000044762.39452.e3. PMID: 12447104
Advances in biology and treatment of childhood brain tumors.
Reddy AT
Curr Neurol Neurosci Rep 2001 Mar;1(2):137-43. doi: 10.1007/s11910-001-0009-7. PMID: 11898509
Pathology, treatment and management of posterior fossa brain tumors in childhood.
Bonner K, Siegel KR
J Neurosci Nurs 1988 Apr;20(2):84-93. doi: 10.1097/01376517-198804000-00004. PMID: 2966218

Prognosis

Supratentorial CNS-PNETs in children; a Swedish population-based study with molecular re-evaluation and long-term follow-up.
Schepke E, Löfgren M, Pietsch T, Kling T, Nordborg C, Olsson Bontell T, Holm S, Öberg A, Nyman P, Eliasson-Hofvander M, Sabel M, Lannering B, Carén H
Clin Epigenetics 2023 Mar 9;15(1):40. doi: 10.1186/s13148-023-01456-2. PMID: 36895035Free PMC Article
Significance and therapeutic value of miRNAs in embryonal neural tumors.
Shalaby T, Fiaschetti G, Baumgartner M, Grotzer MA
Molecules 2014 May 6;19(5):5821-62. doi: 10.3390/molecules19055821. PMID: 24806581Free PMC Article
Recent advances in paediatric neuro-oncology.
Saran F
Curr Opin Neurol 2002 Dec;15(6):671-7. doi: 10.1097/01.wco.0000044762.39452.e3. PMID: 12447104
Childhood brain tumors that occupy more than one compartment at presentation. Multiple compartment tumors.
Gilles FH, Leviton A, Hedley-Whyte ET, Sobel E, Tavaré CJ, Sobel RS, Rorke LB
J Neurooncol 1992 Sep;14(1):45-56. doi: 10.1007/BF00170944. PMID: 1469464
Temporal trends among childhood brain tumor biopsies. The Childhood Brain Tumor Consortium.
Gilles FH, Sobel EL, Leviton A, Hedley-Whyte ET, Tavare CJ, Adelman L, Sobel RA
J Neurooncol 1992 Jun;13(2):137-49. doi: 10.1007/BF00172763. PMID: 1432031

Clinical prediction guides

Supratentorial CNS-PNETs in children; a Swedish population-based study with molecular re-evaluation and long-term follow-up.
Schepke E, Löfgren M, Pietsch T, Kling T, Nordborg C, Olsson Bontell T, Holm S, Öberg A, Nyman P, Eliasson-Hofvander M, Sabel M, Lannering B, Carén H
Clin Epigenetics 2023 Mar 9;15(1):40. doi: 10.1186/s13148-023-01456-2. PMID: 36895035Free PMC Article
Oral and dental late effects in long-term survivors of childhood embryonal brain tumors.
Tanem KE, Stensvold E, Wilberg P, Skaare AB, Brandal P, Herlofson BB
Support Care Cancer 2022 Dec;30(12):10233-10241. Epub 2022 Oct 29 doi: 10.1007/s00520-022-07405-8. PMID: 36307656Free PMC Article
Bone mineral density and nutrition in long-term survivors of childhood brain tumors.
Kvammen JA, Stensvold E, Godang K, Bollerslev J, Myklebust TÅ, Brandal P, Henriksen C, Bechensteen AG
Clin Nutr ESPEN 2022 Aug;50:162-169. Epub 2022 Jun 4 doi: 10.1016/j.clnesp.2022.05.025. PMID: 35871919
Neuropsychological functioning in survivors of childhood medulloblastoma/CNS-PNET: The role of secondary medical complications.
Stadskleiv K, Stensvold E, Stokka K, Bechensteen AG, Brandal P
Clin Neuropsychol 2022 Apr;36(3):600-625. Epub 2020 Jul 30 doi: 10.1080/13854046.2020.1794045. PMID: 32729777
Is there a role for high dose chemotherapy with hematopoietic stem cell rescue in patients with relapsed supratentorial PNET?
Raghuram CP, Moreno L, Zacharoulis S
J Neurooncol 2012 Feb;106(3):441-7. Epub 2011 Aug 18 doi: 10.1007/s11060-011-0690-6. PMID: 21850536

Recent systematic reviews

Brain and Brain Stem Necrosis After Reirradiation for Recurrent Childhood Primary Central Nervous System Tumors: A PENTEC Comprehensive Review.
Ajithkumar T, Avanzo M, Yorke E, Tsang DS, Milano MT, Olch AJ, Merchant TE, Dieckmann K, Mahajan A, Fuji H, Paulino AC, Timmermann B, Marks LB, Bentzen SM, Jackson A, Constine LS
Int J Radiat Oncol Biol Phys 2024 Jun 1;119(2):655-668. Epub 2024 Feb 1 doi: 10.1016/j.ijrobp.2023.12.043. PMID: 38300187
Is there a role for high dose chemotherapy with hematopoietic stem cell rescue in patients with relapsed supratentorial PNET?
Raghuram CP, Moreno L, Zacharoulis S
J Neurooncol 2012 Feb;106(3):441-7. Epub 2011 Aug 18 doi: 10.1007/s11060-011-0690-6. PMID: 21850536

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