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Stage 4 Neuroblastoma

MedGen UID:
78866
•Concept ID:
C0278694
•
Neoplastic Process
Synonyms: Disseminated Neuroblastoma; Metastatic neuroblastoma; Stage IV Neuroblastoma
SNOMED CT: Metastatic neuroblastoma (704152002)

Definition

Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs, except as defined for stage 4S. (cancer.gov) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStage 4 Neuroblastoma

Professional guidelines

PubMed

Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Role of surgery in the treatment of patients with high-risk neuroblastoma who have a poor response to induction chemotherapy.
Du L, Liu L, Zhang C, Cai W, Wu Y, Wang J, Lv F
J Pediatr Surg 2014 Apr;49(4):528-33. Epub 2013 Dec 1 doi: 10.1016/j.jpedsurg.2013.11.061. PMID: 24726106
Neuroblastoma: current drug therapy recommendations as part of the total treatment approach.
Berthold F, Hero B
Drugs 2000 Jun;59(6):1261-77. doi: 10.2165/00003495-200059060-00006. PMID: 10882162

Recent clinical studies

Etiology

Kigelia africana inhibits proliferation and induces cell death in stage 4 Neuroblastoma cell lines.
Ahrens S, Appl B, Trochimiuk M, Dücker C, Feixas Serra G, Oliver Grau A, Reinshagen K, Pagerols Raluy L
Biomed Pharmacother 2022 Oct;154:113584. Epub 2022 Aug 24 doi: 10.1016/j.biopha.2022.113584. PMID: 36029541
Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience.
Berlanga P, Pasqualini C, Pötschger U, Sangüesa C, Castellani MR, Cañete A, Luksch R, Elliot M, Schreier G, Kropf M, Morgenstern D, Papadakis V, Ash S, Ruud E, Brock P, Wieczorek A, Kogner P, Trahair T, Ambros P, Boterberg T, Castel V, Valteau-Couanet D, Ladenstein R
Eur J Cancer 2021 Feb;144:1-8. Epub 2020 Dec 11 doi: 10.1016/j.ejca.2020.10.020. PMID: 33316634
Neuroblastoma between 1990 and 2014 in the Netherlands: Increased incidence and improved survival of high-risk neuroblastoma.
Tas ML, Reedijk AMJ, Karim-Kos HE, Kremer LCM, van de Ven CP, Dierselhuis MP, van Eijkelenburg NKA, van Grotel M, Kraal KCJM, Peek AML, Coebergh JWW, Janssens GOR, de Keizer B, de Krijger RR, Pieters R, Tytgat GAM, van Noesel MM
Eur J Cancer 2020 Jan;124:47-55. Epub 2019 Nov 11 doi: 10.1016/j.ejca.2019.09.025. PMID: 31726247
Interleukin 2 with anti-GD2 antibody ch14.18/CHO (dinutuximab beta) in patients with high-risk neuroblastoma (HR-NBL1/SIOPEN): a multicentre, randomised, phase 3 trial.
Ladenstein R, Pötschger U, Valteau-Couanet D, Luksch R, Castel V, Yaniv I, Laureys G, Brock P, Michon JM, Owens C, Trahair T, Chan GCF, Ruud E, Schroeder H, Beck Popovic M, Schreier G, Loibner H, Ambros P, Holmes K, Castellani MR, Gaze MN, Garaventa A, Pearson ADJ, Lode HN
Lancet Oncol 2018 Dec;19(12):1617-1629. Epub 2018 Nov 12 doi: 10.1016/S1470-2045(18)30578-3. PMID: 30442501
Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article

Diagnosis

Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience.
Berlanga P, Pasqualini C, Pötschger U, Sangüesa C, Castellani MR, Cañete A, Luksch R, Elliot M, Schreier G, Kropf M, Morgenstern D, Papadakis V, Ash S, Ruud E, Brock P, Wieczorek A, Kogner P, Trahair T, Ambros P, Boterberg T, Castel V, Valteau-Couanet D, Ladenstein R
Eur J Cancer 2021 Feb;144:1-8. Epub 2020 Dec 11 doi: 10.1016/j.ejca.2020.10.020. PMID: 33316634
Neuroblastoma between 1990 and 2014 in the Netherlands: Increased incidence and improved survival of high-risk neuroblastoma.
Tas ML, Reedijk AMJ, Karim-Kos HE, Kremer LCM, van de Ven CP, Dierselhuis MP, van Eijkelenburg NKA, van Grotel M, Kraal KCJM, Peek AML, Coebergh JWW, Janssens GOR, de Keizer B, de Krijger RR, Pieters R, Tytgat GAM, van Noesel MM
Eur J Cancer 2020 Jan;124:47-55. Epub 2019 Nov 11 doi: 10.1016/j.ejca.2019.09.025. PMID: 31726247
Interleukin 2 with anti-GD2 antibody ch14.18/CHO (dinutuximab beta) in patients with high-risk neuroblastoma (HR-NBL1/SIOPEN): a multicentre, randomised, phase 3 trial.
Ladenstein R, Pötschger U, Valteau-Couanet D, Luksch R, Castel V, Yaniv I, Laureys G, Brock P, Michon JM, Owens C, Trahair T, Chan GCF, Ruud E, Schroeder H, Beck Popovic M, Schreier G, Loibner H, Ambros P, Holmes K, Castellani MR, Gaze MN, Garaventa A, Pearson ADJ, Lode HN
Lancet Oncol 2018 Dec;19(12):1617-1629. Epub 2018 Nov 12 doi: 10.1016/S1470-2045(18)30578-3. PMID: 30442501
Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Survival and late effects in children with stage 4 neuroblastoma.
Perwein T, Lackner H, Sovinz P, Benesch M, Schmidt S, Schwinger W, Urban C
Pediatr Blood Cancer 2011 Oct;57(4):629-35. Epub 2011 Feb 11 doi: 10.1002/pbc.23036. PMID: 21319289

Therapy

Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience.
Berlanga P, Pasqualini C, Pötschger U, Sangüesa C, Castellani MR, Cañete A, Luksch R, Elliot M, Schreier G, Kropf M, Morgenstern D, Papadakis V, Ash S, Ruud E, Brock P, Wieczorek A, Kogner P, Trahair T, Ambros P, Boterberg T, Castel V, Valteau-Couanet D, Ladenstein R
Eur J Cancer 2021 Feb;144:1-8. Epub 2020 Dec 11 doi: 10.1016/j.ejca.2020.10.020. PMID: 33316634
Interleukin 2 with anti-GD2 antibody ch14.18/CHO (dinutuximab beta) in patients with high-risk neuroblastoma (HR-NBL1/SIOPEN): a multicentre, randomised, phase 3 trial.
Ladenstein R, Pötschger U, Valteau-Couanet D, Luksch R, Castel V, Yaniv I, Laureys G, Brock P, Michon JM, Owens C, Trahair T, Chan GCF, Ruud E, Schroeder H, Beck Popovic M, Schreier G, Loibner H, Ambros P, Holmes K, Castellani MR, Gaze MN, Garaventa A, Pearson ADJ, Lode HN
Lancet Oncol 2018 Dec;19(12):1617-1629. Epub 2018 Nov 12 doi: 10.1016/S1470-2045(18)30578-3. PMID: 30442501
Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Does aggressive surgical resection improve survival in advanced stage 3 and 4 neuroblastoma? A systematic review and meta-analysis.
Mullassery D, Farrelly P, Losty PD
Pediatr Hematol Oncol 2014 Nov;31(8):703-16. Epub 2014 Sep 23 doi: 10.3109/08880018.2014.947009. PMID: 25247398
Survival and late effects in children with stage 4 neuroblastoma.
Perwein T, Lackner H, Sovinz P, Benesch M, Schmidt S, Schwinger W, Urban C
Pediatr Blood Cancer 2011 Oct;57(4):629-35. Epub 2011 Feb 11 doi: 10.1002/pbc.23036. PMID: 21319289

Prognosis

Clinical Impact of KIR2DS3 and KIR2DL3 Genes in Neuroblastoma Patients.
Sezgin G, Görüroğlu Öztürk Ö, Özkan A, Küpeli S, Bayram İ
Med Princ Pract 2022;31(6):532-539. Epub 2022 May 10 doi: 10.1159/000524656. PMID: 35537400Free PMC Article
Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience.
Berlanga P, Pasqualini C, Pötschger U, Sangüesa C, Castellani MR, Cañete A, Luksch R, Elliot M, Schreier G, Kropf M, Morgenstern D, Papadakis V, Ash S, Ruud E, Brock P, Wieczorek A, Kogner P, Trahair T, Ambros P, Boterberg T, Castel V, Valteau-Couanet D, Ladenstein R
Eur J Cancer 2021 Feb;144:1-8. Epub 2020 Dec 11 doi: 10.1016/j.ejca.2020.10.020. PMID: 33316634
Neuroblastoma between 1990 and 2014 in the Netherlands: Increased incidence and improved survival of high-risk neuroblastoma.
Tas ML, Reedijk AMJ, Karim-Kos HE, Kremer LCM, van de Ven CP, Dierselhuis MP, van Eijkelenburg NKA, van Grotel M, Kraal KCJM, Peek AML, Coebergh JWW, Janssens GOR, de Keizer B, de Krijger RR, Pieters R, Tytgat GAM, van Noesel MM
Eur J Cancer 2020 Jan;124:47-55. Epub 2019 Nov 11 doi: 10.1016/j.ejca.2019.09.025. PMID: 31726247
Treatment and outcome of adult-onset neuroblastoma.
Suzuki M, Kushner BH, Kramer K, Basu EM, Roberts SS, Hammond WJ, LaQuaglia MP, Wolden SL, Cheung NV, Modak S
Int J Cancer 2018 Sep 1;143(5):1249-1258. Epub 2018 Apr 6 doi: 10.1002/ijc.31399. PMID: 29574715Free PMC Article
Radical Surgery Improves Survival in Patients with Stage 4 Neuroblastoma.
Vollmer K, Gfroerer S, Theilen TM, Bochennek K, Klingebiel T, Rolle U, Fiegel H
World J Surg 2018 Jun;42(6):1877-1884. doi: 10.1007/s00268-017-4340-9. PMID: 29127465

Clinical prediction guides

Improved Outcomes with Induction Chemotherapy Combined with Arsenic Trioxide in Stage 4 Neuroblastoma: A Case Series.
Li Y, Feng C, Chen Y, Huang K, Li C, Xiong X, Li P, Zhou D, Peng X, Weng W, Deng X, Wu Y, Fang J
Technol Cancer Res Treat 2021 Jan-Dec;20:15330338211041454. doi: 10.1177/15330338211041454. PMID: 34569870Free PMC Article
An 18 gene expression-based score classifier predicts the clinical outcome in stage 4 neuroblastoma.
Formicola D, Petrosino G, Lasorsa VA, Pignataro P, Cimmino F, Vetrella S, Longo L, Tonini GP, Oberthuer A, Iolascon A, Fischer M, Capasso M
J Transl Med 2016 May 17;14(1):142. doi: 10.1186/s12967-016-0896-7. PMID: 27188717Free PMC Article
Neuroblastoma mRNAs predict outcome in children with stage 4 neuroblastoma: a European HR-NBL1/SIOPEN study.
Viprey VF, Gregory WM, Corrias MV, Tchirkov A, Swerts K, Vicha A, Dallorso S, Brock P, Luksch R, Valteau-Couanet D, Papadakis V, Laureys G, Pearson AD, Ladenstein R, Burchill SA
J Clin Oncol 2014 Apr 1;32(10):1074-83. Epub 2014 Mar 3 doi: 10.1200/JCO.2013.53.3604. PMID: 24590653
Clinical potentials of methylator phenotype in stage 4 high-risk neuroblastoma: an open challenge.
Banelli B, Merlo DF, Allemanni G, Forlani A, Romani M
PLoS One 2013;8(5):e63253. Epub 2013 May 22 doi: 10.1371/journal.pone.0063253. PMID: 23717404Free PMC Article
Survival and late effects in children with stage 4 neuroblastoma.
Perwein T, Lackner H, Sovinz P, Benesch M, Schmidt S, Schwinger W, Urban C
Pediatr Blood Cancer 2011 Oct;57(4):629-35. Epub 2011 Feb 11 doi: 10.1002/pbc.23036. PMID: 21319289

Recent systematic reviews

Does aggressive surgical resection improve survival in advanced stage 3 and 4 neuroblastoma? A systematic review and meta-analysis.
Mullassery D, Farrelly P, Losty PD
Pediatr Hematol Oncol 2014 Nov;31(8):703-16. Epub 2014 Sep 23 doi: 10.3109/08880018.2014.947009. PMID: 25247398

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