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Pediatric infratentorial ependymoblastoma

MedGen UID:
272462
•Concept ID:
C1332972
•
Neoplastic Process
Synonyms: Childhood Infratentorial Embryonal Tumor with Multilayered Rosettes, C19MC-Altered; childhood infratentorial embryonal tumor with Multilayered Rosettes, C19MC-altered; childhood infratentorial embryonal tumour with Multilayered Rosettes, C19MC-altered; Childhood Infratentorial Ependymoblastoma; childhood infratentorial ependymoblastoma; Pediatric Infratentorial Ependymoblastoma; pediatric infratentorial ependymoblastoma
 
Monarch Initiative: MONDO:0004263

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPediatric infratentorial ependymoblastoma

Professional guidelines

PubMed

Consensus guidelines for the management of posterior fossa tumour for low- and middle-income countries.
Aziz HF, Bajwa MH, Mushtaq N, Bakhshi SK, Siddiqui K, Khan SA, Shakir M, Masroor M, Suleman F, Enam SA; Pakistan Brain Tumour Consortium
J Pak Med Assoc 2024 Mar;74(3 (Supple-3)):S186-S190. doi: 10.47391/JPMA.S3.GNO-22. PMID: 39262080
Cognitive consequences of the treatment of medulloblastoma among children.
Saury JM, Emanuelson I
Pediatr Neurol 2011 Jan;44(1):21-30. doi: 10.1016/j.pediatrneurol.2010.07.004. PMID: 21147383
Longterm treatment results of childhood medulloblastoma by craniospinal irradiation in supine position.
Slampa P, Pavelka Z, Dusek L, Hynkova L, Sterba J, Ondrova B, Princ D, Novotny T, Kostakova S
Neoplasma 2007;54(1):62-7. PMID: 17203894

Recent clinical studies

Etiology

Mapping pediatric brain tumors to their origins in the developing cerebellum.
Okonechnikov K, Joshi P, Sepp M, Leiss K, Sarropoulos I, Murat F, Sill M, Beck P, Chan KC, Korshunov A, Sah F, Deng MY, Sturm D, DeSisto J, Donson AM, Foreman NK, Green AL, Robinson G, Orr BA, Gao Q, Darrow E, Hadley JL, Northcott PA, Gojo J, Kawauchi D, Hovestadt V, Filbin MG, von Deimling A, Zuckermann M, Pajtler KW, Kool M, Jones DTW, Jäger N, Kutscher LM, Kaessmann H, Pfister SM
Neuro Oncol 2023 Oct 3;25(10):1895-1909. doi: 10.1093/neuonc/noad124. PMID: 37534924Free PMC Article
Medulloblastoma: Clinical presentation.
Vinchon M, Leblond P
Neurochirurgie 2021 Feb;67(1):23-27. Epub 2019 Sep 5 doi: 10.1016/j.neuchi.2019.04.006. PMID: 31494131
Cerebellar mutism.
Pitsika M, Tsitouras V
J Neurosurg Pediatr 2013 Dec;12(6):604-14. Epub 2013 Sep 27 doi: 10.3171/2013.8.PEDS13168. PMID: 24073751
Time to diagnosis of brain tumors in children: a single-centre experience.
Molineus A, Boxberger N, Redlich A, Vorwerk P
Pediatr Int 2013 Jun;55(3):305-9. doi: 10.1111/ped.12095. PMID: 23480630
Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma.
Li MH, Bouffet E, Hawkins CE, Squire JA, Huang A
Neurosurg Focus 2005 Nov 15;19(5):E3. doi: 10.3171/foc.2005.19.5.4. PMID: 16398467

Diagnosis

Medulloblastoma: Clinical presentation.
Vinchon M, Leblond P
Neurochirurgie 2021 Feb;67(1):23-27. Epub 2019 Sep 5 doi: 10.1016/j.neuchi.2019.04.006. PMID: 31494131
Ganglioglioma Arising From Desmoplastic Medulloblastoma: A Case Report and Review of Literature.
Valvi S, Ziegler DS
Pediatrics 2017 Mar;139(3) doi: 10.1542/peds.2016-1403. PMID: 28232638
Paediatric intra-axial posterior fossa tumours: pictorial review.
Rasalkar DD, Chu WC, Paunipagar BK, Cheng FW, Li CK
Postgrad Med J 2013 Jan;89(1047):39-46. Epub 2012 Sep 12 doi: 10.1136/postgradmedj-2011-130075. PMID: 22977284
Hypertrophic olivary degeneration in children: four new cases and a review of the literature with an emphasis on the MRI findings.
Sanverdi SE, Oguz KK, Haliloglu G
Br J Radiol 2012 May;85(1013):511-6. Epub 2012 Feb 14 doi: 10.1259/bjr/60727602. PMID: 22337689Free PMC Article
Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma.
Li MH, Bouffet E, Hawkins CE, Squire JA, Huang A
Neurosurg Focus 2005 Nov 15;19(5):E3. doi: 10.3171/foc.2005.19.5.4. PMID: 16398467

Therapy

Direct Administration of Chemotherapy and Other Agents into the Fourth Ventricle to Treat Recurrent Malignant Brain Tumors in Children.
Sandberg DI, Yu B
Adv Tech Stand Neurosurg 2024;53:119-137. doi: 10.1007/978-3-031-67077-0_8. PMID: 39287806
Meta-Analysis of Apparent Diffusion Coefficient in Pediatric Medulloblastoma, Ependymoma, and Pilocytic Astrocytoma.
Dury RJ, Lourdusamy A, Macarthur DC, Peet AC, Auer DP, Grundy RG, Dineen RA
J Magn Reson Imaging 2022 Jul;56(1):147-157. Epub 2021 Nov 29 doi: 10.1002/jmri.28007. PMID: 34842328
High-dose busulfan-thiotepa with autologous stem cell transplantation followed by posterior fossa irradiation in young children with classical or incompletely resected medulloblastoma.
Bergthold G, El Kababri M, Varlet P, Dhermain F, Sainte-Rose C, Raquin MA, Kieffer V, Goma G, Grill J, Valteau-Couanet D, Dufour C
Pediatr Blood Cancer 2014 May;61(5):907-12. Epub 2014 Jan 28 doi: 10.1002/pbc.24954. PMID: 24470384
Cerebellar mutism.
Pitsika M, Tsitouras V
J Neurosurg Pediatr 2013 Dec;12(6):604-14. Epub 2013 Sep 27 doi: 10.3171/2013.8.PEDS13168. PMID: 24073751
Radiation and chemotherapy in pediatric infratentorial tumors.
Mastrangelo R, Marmiroli L, Tornesello MA, Ausili-Cefaro G
Rays 1993 Oct-Dec;18(4):471-85. PMID: 8047664

Prognosis

Postoperative cerebellar mutism syndrome is an acquired autism-like network disturbance.
Suresh H, Morgan BR, Mithani K, Warsi NM, Yan H, Germann J, Boutet A, Loh A, Gouveia FV, Young J, Quon J, Morgado F, Lerch J, Lozano AM, Al-Fatly B, Kühn AA, Laughlin S, Dewan MC, Mabbott D, Gorodetsky C, Bartels U, Huang A, Tabori U, Rutka JT, Drake JM, Kulkarni AV, Dirks P, Taylor MD, Ramaswamy V, Ibrahim GM
Neuro Oncol 2024 May 3;26(5):950-964. doi: 10.1093/neuonc/noad230. PMID: 38079480Free PMC Article
Medulloblastoma: Clinical presentation.
Vinchon M, Leblond P
Neurochirurgie 2021 Feb;67(1):23-27. Epub 2019 Sep 5 doi: 10.1016/j.neuchi.2019.04.006. PMID: 31494131
Cerebellar mutism: the predictive role of preoperative language evaluation.
Bianchi F, Chieffo DPR, Frassanito P, Di Rocco C, Tamburrini G
Childs Nerv Syst 2020 Jun;36(6):1153-1157. Epub 2019 Jun 14 doi: 10.1007/s00381-019-04252-7. PMID: 31201497
Posterior fossa tumors in children: developmental anatomy and diagnostic imaging.
Raybaud C, Ramaswamy V, Taylor MD, Laughlin S
Childs Nerv Syst 2015 Oct;31(10):1661-76. Epub 2015 Sep 9 doi: 10.1007/s00381-015-2834-z. PMID: 26351220
Paediatric intra-axial posterior fossa tumours: pictorial review.
Rasalkar DD, Chu WC, Paunipagar BK, Cheng FW, Li CK
Postgrad Med J 2013 Jan;89(1047):39-46. Epub 2012 Sep 12 doi: 10.1136/postgradmedj-2011-130075. PMID: 22977284

Clinical prediction guides

Risk factors for domain-specific neurocognitive outcome in pediatric survivors of a brain tumor in the posterior fossa-Results of the HIT 2000 trial.
Mynarek M, Rossius A, Guiard A, Ottensmeier H, von Hoff K, Obrecht-Sturm D, Bußenius L, Friedrich C, von Bueren AO, Gerber NU, Traunwieser T, Kortmann RD, Warmuth-Metz M, Bison B, Thomale UW, Krauss J, Pietsch T, Clifford SC, Pfister SM, Sturm D, Sahm F, Tischler T, Rutkowski S
Neuro Oncol 2024 Nov 4;26(11):2113-2124. doi: 10.1093/neuonc/noae092. PMID: 38835160Free PMC Article
Medulloblastoma: Clinical presentation.
Vinchon M, Leblond P
Neurochirurgie 2021 Feb;67(1):23-27. Epub 2019 Sep 5 doi: 10.1016/j.neuchi.2019.04.006. PMID: 31494131
Pediatric Posterior Fossa Medulloblastoma: The Role of Diffusion Imaging in Identifying Molecular Groups.
Reddy N, Ellison DW, Soares BP, Carson KA, Huisman TAGM, Patay Z
J Neuroimaging 2020 Jul;30(4):503-511. Epub 2020 Jun 12 doi: 10.1111/jon.12704. PMID: 32529709
Cerebellar mutism: the predictive role of preoperative language evaluation.
Bianchi F, Chieffo DPR, Frassanito P, Di Rocco C, Tamburrini G
Childs Nerv Syst 2020 Jun;36(6):1153-1157. Epub 2019 Jun 14 doi: 10.1007/s00381-019-04252-7. PMID: 31201497
Use of apparent diffusion coefficient values for diagnosis of pediatric posterior fossa tumors.
Pierce T, Kranz PG, Roth C, Leong D, Wei P, Provenzale JM
Neuroradiol J 2014 Apr;27(2):233-44. Epub 2014 Apr 18 doi: 10.15274/NRJ-2014-10027. PMID: 24750714Free PMC Article

Recent systematic reviews

Meta-Analysis of Apparent Diffusion Coefficient in Pediatric Medulloblastoma, Ependymoma, and Pilocytic Astrocytoma.
Dury RJ, Lourdusamy A, Macarthur DC, Peet AC, Auer DP, Grundy RG, Dineen RA
J Magn Reson Imaging 2022 Jul;56(1):147-157. Epub 2021 Nov 29 doi: 10.1002/jmri.28007. PMID: 34842328

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