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Supratentorial primitive neuroectodermal tumor(PNET)

MedGen UID:
234914
•Concept ID:
C1336538
•
Neoplastic Process
Synonym: PNET
SNOMED CT: Supratentorial PNET (39781001); Supratentorial primitive neuroectodermal tumor (699318007)
 
Monarch Initiative: MONDO:0003145

Definition

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

Professional guidelines

PubMed

Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India.
Biswas A, Mallick S, Purkait S, Roy S, Sarkar C, Bakhshi S, Singh M, Julka PK, Rath GK
Acta Neurochir (Wien) 2015 Jul;157(7):1251-66. Epub 2015 May 20 doi: 10.1007/s00701-015-2444-2. PMID: 25990846
Current treatment approaches for infants with malignant central nervous system tumors.
Lafay-Cousin L, Strother D
Oncologist 2009 Apr;14(4):433-44. Epub 2009 Apr 2 doi: 10.1634/theoncologist.2008-0193. PMID: 19342475
Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors.
Yang HJ, Nam DH, Wang KC, Kim YM, Chi JG, Cho BK
Childs Nerv Syst 1999 Aug;15(8):377-83. doi: 10.1007/s003810050418. PMID: 10447606

Recent clinical studies

Etiology

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
Salvage Radiosurgery for Recurrent Supratentorial Primitive Neuroectodermal Tumors: A Single Institutional Series and Review of the Literature.
Renfrow JJ, Brown DA, Link MJ, Laack NN, Routman DM, Pollock BE, Parney IF
Stereotact Funct Neurosurg 2021;99(5):405-411. Epub 2021 Apr 21 doi: 10.1159/000515544. PMID: 33882507
Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma.
Chen AY, Lee H, Hartman J, Greco C, Ryu JK, O'Donnell R, Boggan J
AJNR Am J Neuroradiol 2005 Jan;26(1):160-2. PMID: 15661719Free PMC Article
Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor.
Lehrnbecher T, Deinlein F, Marx A, Kühl J
J Pediatr Hematol Oncol 2003 Aug;25(8):657-9. doi: 10.1097/00043426-200308000-00016. PMID: 12902924
Second malignancy after treatment of childhood acute myeloid leukemia.
Leung W, Ribeiro RC, Hudson M, Tong X, Srivastava DK, Rubnitz JE, Sandlund JT, Razzouk BI, Evans WE, Pui CH
Leukemia 2001 Jan;15(1):41-5. doi: 10.1038/sj.leu.2401948. PMID: 11243397

Diagnosis

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
Salvage Radiosurgery for Recurrent Supratentorial Primitive Neuroectodermal Tumors: A Single Institutional Series and Review of the Literature.
Renfrow JJ, Brown DA, Link MJ, Laack NN, Routman DM, Pollock BE, Parney IF
Stereotact Funct Neurosurg 2021;99(5):405-411. Epub 2021 Apr 21 doi: 10.1159/000515544. PMID: 33882507
Supratentorial primitive neuroectodermal tumor (sPNET).
Burger PC
Brain Pathol 2006 Jan;16(1):86. doi: 10.1111/j.1750-3639.2006.tb00565.x. PMID: 16612986Free PMC Article
Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma.
Chen AY, Lee H, Hartman J, Greco C, Ryu JK, O'Donnell R, Boggan J
AJNR Am J Neuroradiol 2005 Jan;26(1):160-2. PMID: 15661719Free PMC Article

Therapy

Proton radiation therapy for pediatric medulloblastoma and supratentorial primitive neuroectodermal tumors: outcomes for very young children treated with upfront chemotherapy.
Jimenez RB, Sethi R, Depauw N, Pulsifer MB, Adams J, McBride SM, Ebb D, Fullerton BC, Tarbell NJ, Yock TI, Macdonald SM
Int J Radiat Oncol Biol Phys 2013 Sep 1;87(1):120-6. Epub 2013 Jun 21 doi: 10.1016/j.ijrobp.2013.05.017. PMID: 23790826
Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy.
Jethwa PR, Lee JH, Assina R, Keller IA, Danish SF
J Neurosurg Pediatr 2011 Nov;8(5):468-75. doi: 10.3171/2011.8.PEDS11148. PMID: 22044371
Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials.
Timmermann B, Kortmann RD, Kühl J, Rutkowski S, Meisner C, Pietsch T, Deinlein F, Urban C, Warmuth-Metz M, Bamberg M
J Clin Oncol 2006 Apr 1;24(10):1554-60. doi: 10.1200/JCO.2005.04.8074. PMID: 16575007
Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M
J Neurol 2005 Mar;252(3):291-9. Epub 2005 Feb 23 doi: 10.1007/s00415-005-0560-2. PMID: 16189725
Second malignancy after treatment of childhood acute myeloid leukemia.
Leung W, Ribeiro RC, Hudson M, Tong X, Srivastava DK, Rubnitz JE, Sandlund JT, Razzouk BI, Evans WE, Pui CH
Leukemia 2001 Jan;15(1):41-5. doi: 10.1038/sj.leu.2401948. PMID: 11243397

Prognosis

Radiation-Induced Supratentorial Primitive Neuroectodermal Tumor after Treatment for Non-Hodgkin's Lymphoma.
Sedney CL, Bhatia S, Bafakih F, Paul S, Gyure K
W V Med J 2015 Jul-Aug;111(4):38-41. PMID: 26242031
Post-chemotherapy maturation in supratentorial primitive neuroectodermal tumors.
Lafay-Cousin L, Hader W, Wei XC, Nordal R, Strother D, Hawkins C, Chan JA
Brain Pathol 2014 Mar;24(2):166-72. Epub 2013 Nov 27 doi: 10.1111/bpa.12089. PMID: 24033491Free PMC Article
Aggressive infantile embryonal tumors.
MacDonald TJ
J Child Neurol 2008 Oct;23(10):1195-204. doi: 10.1177/0883073808321769. PMID: 18952586Free PMC Article
Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma.
Chen AY, Lee H, Hartman J, Greco C, Ryu JK, O'Donnell R, Boggan J
AJNR Am J Neuroradiol 2005 Jan;26(1):160-2. PMID: 15661719Free PMC Article
Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor.
Lehrnbecher T, Deinlein F, Marx A, Kühl J
J Pediatr Hematol Oncol 2003 Aug;25(8):657-9. doi: 10.1097/00043426-200308000-00016. PMID: 12902924

Clinical prediction guides

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
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
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
Multiple CDK/CYCLIND genes are amplified in medulloblastoma and supratentorial primitive neuroectodermal brain tumor.
Li M, Lockwood W, Zielenska M, Northcott P, Ra YS, Bouffet E, Yoshimoto M, Rutka JT, Yan H, Taylor MD, Eberhart C, Hawkins CE, Lam W, Squire JA, Huang A
Cancer Genet 2012 May;205(5):220-31. doi: 10.1016/j.cancergen.2012.03.002. PMID: 22682621

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