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Cerebral primitive neuroectodermal tumor

MedGen UID:
155905
•Concept ID:
C0751675
•
Neoplastic Process
Synonym: Cerebral Primitive Neuroectodermal Tumor
 
Monarch Initiative: MONDO:0004209

Definition

A central nervous system embryonal tumor, not otherwise specified arising from the cerebral hemispheres. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCerebral primitive neuroectodermal tumor

Professional guidelines

PubMed

Management of Neuroblastoma in Pediatric Patients.
Croteau N, Nuchtern J, LaQuaglia MP
Surg Oncol Clin N Am 2021 Apr;30(2):291-304. Epub 2021 Feb 10 doi: 10.1016/j.soc.2020.11.010. PMID: 33706901
Updates in Diagnosis, Management, and Treatment of Neuroblastoma.
Swift CC, Eklund MJ, Kraveka JM, Alazraki AL
Radiographics 2018 Mar-Apr;38(2):566-580. doi: 10.1148/rg.2018170132. PMID: 29528815
Overview and recent advances in the treatment of neuroblastoma.
Whittle SB, Smith V, Doherty E, Zhao S, McCarty S, Zage PE
Expert Rev Anticancer Ther 2017 Apr;17(4):369-386. Epub 2017 Mar 15 doi: 10.1080/14737140.2017.1285230. PMID: 28142287

Recent clinical studies

Etiology

Autologous hematopoietic stem cell transplantation for high-risk brain tumors in children.
Cheuk DK, Lee TL, Chiang AK, Ha SY, Chan GC
J Neurooncol 2008 Feb;86(3):337-47. Epub 2007 Sep 29 doi: 10.1007/s11060-007-9478-0. PMID: 17906911Free PMC Article
Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.
Yates AJ, Franklin TK, McKinney P, Collins R, Comas T, Boesel CP, Pearl DK
J Mol Neurosci 1999 Apr;12(2):111-21. doi: 10.1007/BF02736925. PMID: 10527455
p53 gene mutation in cerebral primitive neuroectodermal tumor in Taiwan.
Ho YS, Hsieh LL, Chen JS, Chang CN, Lee ST, Chiu LL, Chin TY, Cheng SC
Cancer Lett 1996 Jun 24;104(1):103-13. doi: 10.1016/0304-3835(96)04238-3. PMID: 8640736
A midline cerebral primitive neuroectodermal tumor: immunohistochemical evidence for a germinal matrix origin.
Blaauwgeers JL, Troost D
Clin Neuropathol 1988 Mar-Apr;7(2):73-6. PMID: 2839313
Preradiation chemotherapy for newly diagnosed childhood brain tumors. A modified Phase II trial.
Allen JC, Helson L, Jereb B
Cancer 1983 Dec 1;52(11):2001-6. doi: 10.1002/1097-0142(19831201)52:11<2001::aid-cncr2820521105>3.0.co;2-d. PMID: 6627214

Diagnosis

Primary diffuse leptomeningeal primitive neuroectodermal tumor presenting as chronic meningitis.
Koshy KG, Kulkarni GB, Nandeesh BN, Taalapalli AV
J Postgrad Med 2024 Jan-Mar;70(1):46-49. doi: 10.4103/jpgm.jpgm_495_22. PMID: 36891942Free PMC Article
Tumor disease and associated congenital abnormalities on prenatal MRI.
Nemec SF, Horcher E, Kasprian G, Brugger PC, Bettelheim D, Amann G, Nemec U, Rotmensch S, Rimoin DL, Graham JM Jr, Prayer D
Eur J Radiol 2012 Feb;81(2):e115-22. Epub 2011 Feb 12 doi: 10.1016/j.ejrad.2010.12.095. PMID: 21316891
Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation.
Jeans AF, Frayling I, Jasani B, Side L, Blesing C, Ansorge O
Nat Rev Clin Oncol 2009 May;6(5):295-9. doi: 10.1038/nrclinonc.2009.35. PMID: 19390556
Neonatal macrocephaly: cerebral primitive neuroectodermal tumor or neuroblastoma as an infrequent cause--a case report and review of the literature.
Kaczala GW, Poskitt KJ, Steinbok P, Hendson G, Eydoux P, Solimano AJ
Am J Perinatol 2007 Oct;24(9):507-9. Epub 2007 Oct 1 doi: 10.1055/s-2007-986698. PMID: 17909991
Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.
Yates AJ, Franklin TK, McKinney P, Collins R, Comas T, Boesel CP, Pearl DK
J Mol Neurosci 1999 Apr;12(2):111-21. doi: 10.1007/BF02736925. PMID: 10527455

Therapy

Autologous hematopoietic stem cell transplantation for high-risk brain tumors in children.
Cheuk DK, Lee TL, Chiang AK, Ha SY, Chan GC
J Neurooncol 2008 Feb;86(3):337-47. Epub 2007 Sep 29 doi: 10.1007/s11060-007-9478-0. PMID: 17906911Free PMC Article
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361
Human interferon-beta therapy for cerebral primitive neuroectodermal tumors--two case reports.
Tanaka S, Okada M, Murakami M, Hirakawa M, Manaka S, Nagashima T
Neurol Med Chir (Tokyo) 1995 Feb;35(2):82-6. doi: 10.2176/nmc.35.82. PMID: 7753313
Neuraxis dissemination in pediatric brain tumors. Response to preirradiation chemotherapy.
Kellie SJ, Kovnar EH, Kun LE, Horowitz ME, Heideman RL, Douglass EC, Langston JW, Sanford RA, Jenkins JJ 3rd, Fairclough DL
Cancer 1992 Feb 15;69(4):1061-6. doi: 10.1002/1097-0142(19920215)69:4<1061::aid-cncr2820690438>3.0.co;2-8. PMID: 1735073
Preradiation chemotherapy for newly diagnosed childhood brain tumors. A modified Phase II trial.
Allen JC, Helson L, Jereb B
Cancer 1983 Dec 1;52(11):2001-6. doi: 10.1002/1097-0142(19831201)52:11<2001::aid-cncr2820521105>3.0.co;2-d. PMID: 6627214

Prognosis

Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation.
Jeans AF, Frayling I, Jasani B, Side L, Blesing C, Ansorge O
Nat Rev Clin Oncol 2009 May;6(5):295-9. doi: 10.1038/nrclinonc.2009.35. PMID: 19390556
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361
Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.
Yates AJ, Franklin TK, McKinney P, Collins R, Comas T, Boesel CP, Pearl DK
J Mol Neurosci 1999 Apr;12(2):111-21. doi: 10.1007/BF02736925. PMID: 10527455
Cerebral primitive neuroectodermal tumor in an adult, with spinal cord metastasis after 18-year dormancy.
Louis DN, Hochberg FH
J Neurooncol 1990 Aug;9(1):77-80. doi: 10.1007/BF00167072. PMID: 2170592
Preradiation chemotherapy for newly diagnosed childhood brain tumors. A modified Phase II trial.
Allen JC, Helson L, Jereb B
Cancer 1983 Dec 1;52(11):2001-6. doi: 10.1002/1097-0142(19831201)52:11<2001::aid-cncr2820521105>3.0.co;2-d. PMID: 6627214

Clinical prediction guides

Autologous hematopoietic stem cell transplantation for high-risk brain tumors in children.
Cheuk DK, Lee TL, Chiang AK, Ha SY, Chan GC
J Neurooncol 2008 Feb;86(3):337-47. Epub 2007 Sep 29 doi: 10.1007/s11060-007-9478-0. PMID: 17906911Free PMC Article
Temozolomide in resistant or relapsed pediatric solid tumors.
De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A
Pediatr Blood Cancer 2006 Jul;47(1):30-6. doi: 10.1002/pbc.20516. PMID: 16047361
Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.
Yates AJ, Franklin TK, McKinney P, Collins R, Comas T, Boesel CP, Pearl DK
J Mol Neurosci 1999 Apr;12(2):111-21. doi: 10.1007/BF02736925. PMID: 10527455
Ultrastructural study of nuclear evagination in neoplastic cells of a cerebral primitive neuroectodermal tumor.
Ho KL
Clin Neuropathol 1989 May-Jun;8(3):142-6. PMID: 2545397

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