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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
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
Treatment of neuroblastoma stage 4 with 131I-meta-iodo-benzylguanidine, high-dose chemotherapy and immunotherapy. A pilot study.
Klingebiel T, Bader P, Bares R, Beck J, Hero B, Jürgens H, Lang P, Niethammer D, Rath B, Handgretinger R
Eur J Cancer 1998 Aug;34(9):1398-402. doi: 10.1016/s0959-8049(98)00130-0. PMID: 9849423

Recent clinical studies

Etiology

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
Brain metastasis in children with stage 4 neuroblastoma after multidisciplinary treatment.
Zhu J, Wang J, Zhen ZJ, Lu SY, Zhang F, Sun FF, Li PF, Huang JT, Cai RQ, Sun XF
Chin J Cancer 2015 Sep 14;34(11):531-7. doi: 10.1186/s40880-015-0038-2. PMID: 26370236Free 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

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
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
Brain metastasis in children with stage 4 neuroblastoma after multidisciplinary treatment.
Zhu J, Wang J, Zhen ZJ, Lu SY, Zhang F, Sun FF, Li PF, Huang JT, Cai RQ, Sun XF
Chin J Cancer 2015 Sep 14;34(11):531-7. doi: 10.1186/s40880-015-0038-2. PMID: 26370236Free 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

Randomized Trial of Two Induction Therapy Regimens for High-Risk Neuroblastoma: HR-NBL1.5 International Society of Pediatric Oncology European Neuroblastoma Group Study.
Garaventa A, Poetschger U, Valteau-Couanet D, Luksch R, Castel V, Elliott M, Ash S, Chan GCF, Laureys G, Beck-Popovic M, Vettenranta K, Balwierz W, Schroeder H, Owens C, Cesen M, Papadakis V, Trahair T, Schleiermacher G, Ambros P, Sorrentino S, Pearson ADJ, Ladenstein RL
J Clin Oncol 2021 Aug 10;39(23):2552-2563. Epub 2021 Jun 21 doi: 10.1200/JCO.20.03144. PMID: 34152804
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
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
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
Brain metastasis in children with stage 4 neuroblastoma after multidisciplinary treatment.
Zhu J, Wang J, Zhen ZJ, Lu SY, Zhang F, Sun FF, Li PF, Huang JT, Cai RQ, Sun XF
Chin J Cancer 2015 Sep 14;34(11):531-7. doi: 10.1186/s40880-015-0038-2. PMID: 26370236Free 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

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
Randomized Trial of Two Induction Therapy Regimens for High-Risk Neuroblastoma: HR-NBL1.5 International Society of Pediatric Oncology European Neuroblastoma Group Study.
Garaventa A, Poetschger U, Valteau-Couanet D, Luksch R, Castel V, Elliott M, Ash S, Chan GCF, Laureys G, Beck-Popovic M, Vettenranta K, Balwierz W, Schroeder H, Owens C, Cesen M, Papadakis V, Trahair T, Schleiermacher G, Ambros P, Sorrentino S, Pearson ADJ, Ladenstein RL
J Clin Oncol 2021 Aug 10;39(23):2552-2563. Epub 2021 Jun 21 doi: 10.1200/JCO.20.03144. PMID: 34152804
Establishment and characterization of new orthotopic and metastatic neuroblastoma models.
Daudigeos-Dubus E, LE Dret L, Rouffiac V, Bawa O, Leguerney I, Opolon P, Vassal G, Geoerger B
In Vivo 2014 Jul-Aug;28(4):425-34. PMID: 24982206
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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