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Localized Primitive Neuroectodermal Tumor

MedGen UID:
272797
•Concept ID:
C1334410
•
Neoplastic Process

Definition

A primitive neuroectodermal tumor that is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Professional guidelines

PubMed

Bispecific antibodies for the treatment of neuroblastoma.
Espinosa-Cotton M, Cheung NV
Pharmacol Ther 2022 Sep;237:108241. Epub 2022 Jul 10 doi: 10.1016/j.pharmthera.2022.108241. PMID: 35830901Free PMC Article
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
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

Cellular senescence in neuroblastoma.
Zanotti S, Decaesteker B, Vanhauwaert S, De Wilde B, De Vos WH, Speleman F
Br J Cancer 2022 Jun;126(11):1529-1538. Epub 2022 Feb 23 doi: 10.1038/s41416-022-01755-0. PMID: 35197583Free PMC Article
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
Neuroblastoma.
Davidoff AM
Semin Pediatr Surg 2012 Feb;21(1):2-14. doi: 10.1053/j.sempedsurg.2011.10.009. PMID: 22248965Free PMC Article
Recent advances in neuroblastoma.
Maris JM
N Engl J Med 2010 Jun 10;362(23):2202-11. doi: 10.1056/NEJMra0804577. PMID: 20558371Free PMC Article
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171

Diagnosis

Amplification of the PLAG-family genes-PLAGL1 and PLAGL2-is a key feature of the novel tumor type CNS embryonal tumor with PLAGL amplification.
Keck MK, Sill M, Wittmann A, Joshi P, Stichel D, Beck P, Okonechnikow K, Sievers P, Wefers AK, Roncaroli F, Avula S, McCabe MG, Hayden JT, Wesseling P, Øra I, Nistér M, Kranendonk MEG, Tops BBJ, Zapotocky M, Zamecnik J, Vasiljevic A, Fenouil T, Meyronet D, von Hoff K, Schüller U, Loiseau H, Figarella-Branger D, Kramm CM, Sturm D, Scheie D, Rauramaa T, Pesola J, Gojo J, Haberler C, Brandner S, Jacques T, Sexton Oates A, Saffery R, Koscielniak E, Baker SJ, Yip S, Snuderl M, Ud Din N, Samuel D, Schramm K, Blattner-Johnson M, Selt F, Ecker J, Milde T, von Deimling A, Korshunov A, Perry A, Pfister SM, Sahm F, Solomon DA, Jones DTW
Acta Neuropathol 2023 Jan;145(1):49-69. Epub 2022 Nov 27 doi: 10.1007/s00401-022-02516-2. PMID: 36437415Free PMC Article
Melanotic Neuroectodermal Tumor of Infancy.
Soles BS, Wilson A, Lucas DR, Heider A
Arch Pathol Lab Med 2018 Nov;142(11):1358-1363. doi: 10.5858/arpa.2018-0241-RA. PMID: 30407852
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
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171
Neuroblastoma.
Humpl T
World J Urol 1995;13(4):233-9. doi: 10.1007/BF00182969. PMID: 8528298

Therapy

Cancer-selective metabolic vulnerabilities in MYC-amplified medulloblastoma.
Gwynne WD, Suk Y, Custers S, Mikolajewicz N, Chan JK, Zador Z, Chafe SC, Zhai K, Escudero L, Zhang C, Zaslaver O, Chokshi C, Shaikh MV, Bakhshinyan D, Burns I, Chaudhry I, Nachmani O, Mobilio D, Maich WT, Mero P, Brown KR, Quaile AT, Venugopal C, Moffat J, Montenegro-Burke JR, Singh SK
Cancer Cell 2022 Dec 12;40(12):1488-1502.e7. Epub 2022 Nov 10 doi: 10.1016/j.ccell.2022.10.009. PMID: 36368321
Advancing therapy for neuroblastoma.
Qiu B, Matthay KK
Nat Rev Clin Oncol 2022 Aug;19(8):515-533. Epub 2022 May 25 doi: 10.1038/s41571-022-00643-z. PMID: 35614230
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
Neuroblastoma.
Davidoff AM
Semin Pediatr Surg 2012 Feb;21(1):2-14. doi: 10.1053/j.sempedsurg.2011.10.009. PMID: 22248965Free PMC Article
Recent advances in neuroblastoma.
Maris JM
N Engl J Med 2010 Jun 10;362(23):2202-11. doi: 10.1056/NEJMra0804577. PMID: 20558371Free PMC Article

Prognosis

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
Embryonal brain tumors.
McGovern SL, Grosshans D, Mahajan A
Cancer J 2014 Nov-Dec;20(6):397-402. doi: 10.1097/PPO.0000000000000081. PMID: 25415685
Recent advances in neuroblastoma.
Maris JM
N Engl J Med 2010 Jun 10;362(23):2202-11. doi: 10.1056/NEJMra0804577. PMID: 20558371Free PMC Article
Retroperitoneal sarcomas.
Eilber FC, Eilber KS, Eilber FR
Curr Treat Options Oncol 2000 Aug;1(3):274-8. doi: 10.1007/s11864-000-0040-y. PMID: 12057171
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

Molecular classification and outcome of children with rare CNS embryonal tumors: results from St. Jude Children's Research Hospital including the multi-center SJYC07 and SJMB03 clinical trials.
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free PMC Article
Cellular senescence in neuroblastoma.
Zanotti S, Decaesteker B, Vanhauwaert S, De Wilde B, De Vos WH, Speleman F
Br J Cancer 2022 Jun;126(11):1529-1538. Epub 2022 Feb 23 doi: 10.1038/s41416-022-01755-0. PMID: 35197583Free PMC Article
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
Neuroblastoma: clinical and biological approach to risk stratification and treatment.
Tolbert VP, Matthay KK
Cell Tissue Res 2018 May;372(2):195-209. Epub 2018 Mar 23 doi: 10.1007/s00441-018-2821-2. PMID: 29572647Free PMC Article
Neuroblastoma.
Davidoff AM
Semin Pediatr Surg 2012 Feb;21(1):2-14. doi: 10.1053/j.sempedsurg.2011.10.009. PMID: 22248965Free PMC Article

Recent systematic reviews

Prognostic value of CT characteristics in GEP-NET: A systematic review.
van der Velden DL, Staal FCR, Aalbersberg EA, Castagnoli F, Wilthagen E, Beets-Tan RGH
Crit Rev Oncol Hematol 2022 Jul;175:103713. Epub 2022 May 20 doi: 10.1016/j.critrevonc.2022.103713. PMID: 35598829
Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.
Cherif El Asri A, Benzagmout M, Chakour K, Chaoui MF, Laaguili J, Chahdi H, Gazzaz M, El Mostarchid B
World Neurosurg 2018 Jul;115:346-356. Epub 2018 May 3 doi: 10.1016/j.wneu.2018.04.164. PMID: 29729469
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
Minimally invasive adrenalectomy in children.
Heloury Y, Muthucumaru M, Panabokke G, Cheng W, Kimber C, Leclair MD
J Pediatr Surg 2012 Feb;47(2):415-21. doi: 10.1016/j.jpedsurg.2011.08.003. PMID: 22325405
High-dose chemotherapy and autologous haematopoietic stem cell rescue for children with high-risk neuroblastoma.
Yalçin B, Kremer LC, Caron HN, van Dalen EC
Cochrane Database Syst Rev 2010 May 12;(5):CD006301. doi: 10.1002/14651858.CD006301.pub2. PMID: 20464740

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