Skip to main content

Neuroblastoma

  • Reference work entry
  • First Online:
Pediatric Surgery
  • 1305 Accesses

Abstract

Neuroblastoma is a heterogeneous disease; tumors can spontaneously regress or mature or display an aggressive, therapy-resistant phenotype. Increasing evidence indicates that the biologic and molecular features of neuroblastoma significantly influence and are highly predictive of clinical behavior. Because of this, neuroblastoma has served as a paradigm for biological risk assessment and treatment assignment. Most current clinical studies of neuroblastoma base therapy and its intensity on a risk stratification that takes into account both clinical and biologic variables predictive of relapse. For example, surgery alone offers definitive therapy with excellent outcome for patients with low-risk disease, while patients at high risk of disease relapse are treated with intensive multimodality therapy. Further refinements in the risk assignment algorithm and the development of targeted therapies based on an increased understanding of the molecular pathogenesis of neuroblastoma continue to be investigated.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

Similar content being viewed by others

References

  • Adams GA, Shochat SJ, Smith EI, Shuster JJ, Joshi VV, Altshuler G, et al. Thoracic neuroblastoma: a Pediatric Oncology Group study. J Pediatr Surg. 1993;28(3):372–7.

    Article  CAS  PubMed  Google Scholar 

  • Adkins ES, Sawin R, Gerbing RB, London WB, Matthay KK, Haase GM. Efficacy of complete resection for high-risk neuroblastoma: a Children’s Cancer Group study. J Pediatr Surg. 2004;39:931–6.

    Article  PubMed  Google Scholar 

  • Attiyeh EF, London WB, Mosse YP, Wang Q, Winter C, Khazi D, et al. Chromosome 1p and 11q deletions and outcome in neuroblastoma. N Engl J Med. 2005;353(21):2243–53.

    Article  CAS  PubMed  Google Scholar 

  • Azizkhan RG, Shaw A, Chandler JG. Surgical complications of neuroblastoma resection. Surgery. 1985;97(5):514–7.

    CAS  PubMed  Google Scholar 

  • Baker DL, Schmidt ML, Cohn SL, Maris JM, London WB, Buxton A, et al. Outcome after reduced chemotherapy for intermediate-risk neuroblastoma. N Engl J Med. 2010;363(14):1313–23.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Barr EK, Applebaum MA. Genetic predisposition to neuroblastoma. Children (Basel). 2018;5(9):119.

    Google Scholar 

  • Beckwith JB, Perrin EV. In situ neuroblastoma: a contribution to the natural history of neural crest tumors. Am J Pathol. 1963;43:1089.

    CAS  PubMed  PubMed Central  Google Scholar 

  • Brodeur GM, Seeger RC, Schwab M, Varmus HE, Bishop JM. Amplification of N-myc in untreated human neuroblastomas correlates with advanced disease stage. Science. 1984;224(4653):1121–4.

    Article  CAS  PubMed  Google Scholar 

  • Brodeur GM, Hayes FA, Green AA, Casper JT, Wasson J, Wallach S, et al. Consistent N-myc copy number in simultaneous or consecutive neuroblastoma samples from sixty individual patients. Cancer Res. 1987;47(16):4248–53.

    CAS  PubMed  Google Scholar 

  • Brodeur GM, Pritchard J, Berthold F, Carlsen NL, Castel V, Castelberry RP, et al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J Clin Oncol. 1993;11(8):1466–77.

    Article  CAS  PubMed  Google Scholar 

  • Brodeur GM, Hogarty MD, Mosse YP, Maris JM. Neuroblastoma. In: Pizzo PA, Poplack DG, editors. Principles and practice of pediatric oncology. 6th ed. Philadelphia: Lippincott; 2010.

    Google Scholar 

  • Carpenter EL, Mosse YP. Targeting ALK in neuroblastoma-preclinical and clinical advancements. Nat Rev Clin Oncol. 2012;9(7):391–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Castel V, Tovar JA, Costa E, Cuadros J, Ruiz A, Rollan V, et al. The role of surgery in stage IV neuroblastoma. J Pediatr Surg. 2002;37(11):1574–8.

    Article  PubMed  Google Scholar 

  • Cecchetto G, Mosseri V, De Bernardi B, Helardot P, Monclair T, Costa E, et al. Surgical risk factors in primary surgery for localized neuroblastoma: the LNESG1 study of the European International Society of Pediatric Oncology Neuroblastoma Group. J Clin Oncol. 2005;23(33):8483–9.

    Article  PubMed  Google Scholar 

  • Cheung NK, Zhang J, Lu C, Parker M, Bahrami A, Tickoo SK, et al. Association of age at diagnosis and genetic mutations in patients with neuroblastoma. JAMA. 2012;307(10):1062–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Cohn SL, Pearson AD, London WB, Monclair T, Ambros PF, Brodeur GM, et al. The international neuroblastoma risk group (INRG) classification system: an INRG Task Force report. J Clin Oncol. 2009;27(2):289–97.

    Article  PubMed  PubMed Central  Google Scholar 

  • D’Angio GJ, Evans AE, Koop CE. Special pattern of widespread neuroblastoma with a favourable prognosis. Lancet. 1971;1(7708):1046–9.

    Article  PubMed  Google Scholar 

  • De BB, Gambini C, Haupt R, Granata C, Rizzo A, Conte M, et al. Retrospective study of childhood ganglioneuroma. J Clin Oncol. 2008;26(10):1710–6.

    Article  Google Scholar 

  • Gelfand MJ. Meta-iodobenzylguanidine in children. Semin Nucl Med. 1993;23(3):231–42.

    Article  CAS  PubMed  Google Scholar 

  • Gosfeld JL, et al. Neuroblastoma: an analysis of 160 cases. World J Surg. 1980;4:29–38.

    Article  Google Scholar 

  • Kaneko Y, Kanda N, Maseki N, Sakurai M, Tsuchida Y, Takeda T, et al. Different karyotypic patterns in early and advanced stage neuroblastomas. Cancer Res. 1987;47(1):311–8.

    CAS  PubMed  Google Scholar 

  • Katzenstein HM, Kent PM, London WB, Cohn SL. Treatment and outcome of 83 children with intraspinal neuroblastoma: the Pediatric Oncology Group experience. J Clin Oncol. 2001;19(4):1047–55.

    Article  CAS  PubMed  Google Scholar 

  • Kiely EM. The surgical challenge of neuroblastoma. J Pediatr Surg. 1994;29(2):128–33.

    Article  CAS  PubMed  Google Scholar 

  • Kuroda T, Saeki M, Honna T, Masaki H, Tsunematsu Y. Clinical significance of intensive surgery with intraoperative radiation for advanced neuroblastomas: does it really make sense? J Pediatr Surg. 2003;38:1735–8.

    Article  PubMed  Google Scholar 

  • La Quaglia MP, Kushner BH, Heller G, Bonilla MA, Lindsley KL, Cheung NK. Stage 4 neuroblastoma diagnosed at more than 1 year of age: gross total resection and clinical outcome. J Pediatr Surg. 1994;29(8):1162–5.

    Article  PubMed  Google Scholar 

  • La Quaglia MP, Kushner BH, Su W, et al. The impact of gross total resection on local control and survival in high risk neuroblastoma. J Pediatr Surg. 2004;39(3):412–7.

    Google Scholar 

  • Lashford LS, Lewis IJ, Fielding SL, Flower MA, Meller S, Kemshead JT, et al. Phase I/II study of iodine 131 metaiodobenzylguanidine in chemoresistant neuroblastoma: a United Kingdom Children’s Cancer Study Group investigation. J Clin Oncol. 1992;10(12):1889–96.

    Article  CAS  PubMed  Google Scholar 

  • Look AT, Hayes FA, Nitschke R, McWilliams NB, Green AA. Cellular DNA content as a predictor of response to chemotherapy in infants with unresectable neuroblastoma. N Engl J Med. 1984;311(4):231–5.

    Article  CAS  PubMed  Google Scholar 

  • Medary I, Aronson D, Cheung NK, Ghavimi F, Gerald W, La Quaglia MP. Kinetics of primary tumor regression with chemotherapy: implications for the timing of surgery. Ann Surg Oncol. 1996;3(6):521–5.

    Article  CAS  PubMed  Google Scholar 

  • Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K, et al. The international neuroblastoma risk group (INRG) staging system: an INRG Task Force report. J Clin Oncol. 2009;27(2):298–303.

    Article  PubMed  PubMed Central  Google Scholar 

  • Mosse YP, Laudenslager M, Longo L, Cole KA, Wood A, Attiyeh EF, et al. Identification of ALK as a major familial neuroblastoma predisposition gene. Nature. 2008;455(7215):930–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Nuchtern JG, London WB, Barnewolt CE, Naranjo A, McGrady PW, Geiger JD, et al. A prospective study of expectant observation as primary therapy for neuroblastoma in young infants: a Children’s Oncology Group study. Ann Surg. 2012;256(4):573–80.

    Article  PubMed  Google Scholar 

  • Perez CA, Matthay KK, Atkinson JB, Seeger RC, Shimada H, Haase GM, et al. Biologic variables in the outcome of stages I and II neuroblastoma treated with surgery as primary therapy: a children’s cancer group study. J Clin Oncol. 2000;18(1):18–26.

    Article  CAS  PubMed  Google Scholar 

  • Peuchmaur M, d’Amore ES, Joshi VV, Hata J, Roald B, Dehner LP, et al. Revision of the international neuroblastoma pathology classification: confirmation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer. 2003;98(10):2274–81.

    Article  PubMed  Google Scholar 

  • Sano H, Bonadio J, Gerbing RB, London WB, Matthay KK, Lukens JN, et al. International neuroblastoma pathology classification adds independent prognostic information beyond the prognostic contribution of age. Eur J Cancer. 2006;42(8):1113–9.

    Article  PubMed  Google Scholar 

  • Schilling FH, Spix C, Berthold F, Erttmann R, Fehse N, Hero B, et al. Neuroblastoma screening at one year of age. N Engl J Med. 2002;346(14):1047–53.

    Article  PubMed  Google Scholar 

  • Schmidt ML, Lundblad D, Seeger RC, Brodeur GM, Shimada H, Gerbing RB. Biologic factors determine prognosis in infants with stage IV neuroblastoma: a prospective Children’s Cancer Group study. J Clin Oncol. 2000;18(6):1260–8.

    Article  CAS  PubMed  Google Scholar 

  • Seeger RC, Brodeur GM, Sather H, Dalton A, Siegel SE, Wong KY, et al. Association of multiple copies of the N-myc oncogene with rapid progression of neuroblastomas. N Engl J Med. 1985;313(18):1111–6.

    Article  CAS  PubMed  Google Scholar 

  • Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B, et al. The international neuroblastoma pathology classification (the Shimada system). Cancer. 1999;86(2):364–72.

    Article  CAS  PubMed  Google Scholar 

  • Shimada H, Umehara S, Monobe Y, Hachitanda Y, Nakagawa A, Goto S, et al. International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children’s Cancer Group. Cancer. 2001;92(9):2451–61.

    Article  CAS  PubMed  Google Scholar 

  • Shorter NA, Davidoff AM, Evans AE, Ross AJ III, Zeigler MM, O’Neill JA Jr. The role of surgery in the management of stage IV neuroblastoma: a single institution study. Med Pediatr Oncol. 1995;24(5):287–91.

    Article  CAS  PubMed  Google Scholar 

  • Siegel MJ, Ishwaran H, Fletcher BD, Meyer JS, Hoffer FA, Jaramillo D, et al. Staging of neuroblastoma at imaging: report of the radiology diagnostic oncology group. Radiology. 2002;223(1):168–75.

    Article  PubMed  Google Scholar 

  • Simon T, Haberle B, Hero B, von Schweintiz D, Berthold F. Role of surgery in the treatment of patients with stage 4 neuroblastoma age 18 months or older at diagnosis. J Clin Oncol. 2013;31(6):752–8.

    Article  PubMed  Google Scholar 

  • Stark DD, Moss AA, Brasch RC, de Lorimier AA, Albin AR, London DA, et al. Neuroblastoma: diagnostic imaging and staging. Radiology. 1983;148(1):101–5.

    Article  CAS  PubMed  Google Scholar 

  • Strother DR, London WB, Schmidt ML, Brodeur GM, Shimada H, Thorner P, et al. Outcome after surgery alone or with restricted use of chemotherapy for patients with low-risk neuroblastoma: results of Children’s Oncology Group study P9641. J Clin Oncol. 2012;30(15):1842–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Swift CC, Eklund MJ, Kraveka JM, et al. Updates in diagnosis, management, and treatment of neuroblastoma. Radiographics. 2018;38(2):566–80.

    Article  PubMed  Google Scholar 

  • Voeller J, Sondel PM. Advances in anti-GD2 immunotherapy for treatment of high-risk neuroblastoma. J Pediatr Hematol Oncol. 2019;41(3):163–9.

    Article  PubMed  PubMed Central  Google Scholar 

  • von Schweinitz D, Hero B, Berthold F. The impact of surgical radicality on outcome in childhood neuroblastoma. Eur J Pediatr Surg. 2002;12(6):402–9.

    Article  Google Scholar 

  • Woods WG, Gao RN, Shuster JJ, Robison LL, Bernstein M, Weitzman S, et al. Screening of infants and mortality due to neuroblastoma. N Engl J Med. 2002;346(14):1041–6.

    Article  PubMed  Google Scholar 

  • Yamamoto K, Hanada R, Kikuchi A, Ichikawa M, Aihara T, Oguma E, et al. Spontaneous regression of localized neuroblastoma detected by mass screening. J Clin Oncol. 1998;16(4):1265–9.

    Article  CAS  PubMed  Google Scholar 

  • Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, et al. Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma. N Engl J Med. 2010;363(14):1324–34.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgments

This chapter has been adapted from the author’s own chapters in the following publications: Seminars in Pediatric Surgery (Volume 21, Issue 1, February 2012). Copyright © 2012 Elsevier Inc. and Ashcraft’s Pediatric Surgery (Fifth edition) edited by George Whitfield Holcomb, III, J. Patrick Murphy and Daniel J. Ostlie. Copyright © 2010 Elsevier Inc.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Andrew M. Davidoff .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer-Verlag GmbH Germany, part of Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Davidoff, A.M. (2021). Neuroblastoma. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43559-5_146

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-43559-5_146

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-43558-8

  • Online ISBN: 978-3-662-43559-5

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics