Abstract
Neuroblastoma represents 10% of childhood solid tumours, with an incidence of 6–8 per million. The tumours develop in cells derived from the neural crest and may arise in any structure with the crest as its origin. There is an extra-ordinary variation in the presentation and outcome of the disease with some patients dying from extensive metastases in spite of intensive multimodal therapy and others experiencing disappearance of the tumour with no treatment.
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Willis RA. Pathology of tumours. 4th ed. London: Butterworth& Co; 1967. pp. 857–60.
Pepper W. A study of congenital sarcoma of the liver and suprarenal. Am J Med Sci. 1901;121:287–99.
Wright JH. Neurocytoma or neuroblastoma, a kind of tumour not generally recognised. J Exp Med. 1910;12:556–61.
Lehman EP. Adrenal neuroblastoma in infancy—15 year survival. Ann Surg. 1932;95:473.
Blacklock JWS. Neurogenic tumours of the sympathetic system in children. J Pathol Bacteriol. 1934;39:27–48.
Wittenborg MH. Roentgen therapy in neuroblastoma. A review of 73 cases. 1950; 54:679–88.
Gross RE, Farber S, Martin LW. Neuroblastoma sympatheticum: a study and report of 217 cases. Pediatrics. 1959;23:1179–91.
Koop CE, Johnson DG. Neuroblastoma: an assessment of therapy in reference to staging. J Ped Surg. 1971;6:595–600.
Swank RL II, Fetterman GH, Sieber WK, Kiesewetter WB. Prognostic factors in neuroblastoma. Ann Surg. 1971;174:428–35.
Lehman EP. Neuroblastoma. With report of a case. J Med Res. 1917; 36:309–26.
Sutow WW, Gehan EA, Heyn RM, et al. Comparison of survival curves, 1956 versus 1962, in children with Wilms’ tumour and neuroblastoma. Pediatrics. 1970;45:800–11.
Pinkel D, Pratt C, Holton C, et al. Survival of children with neuroblastoma treated with combination chemotherapy. J Pediatr. 1968;73:928–31.
Leikin S, Evans A, Heyn R, Newton W. The impact of chemotherapy on advanced neuroblastoma: survival of patients diagnosed in 1956, 1962 and 1966–68 in Children’s Cancer Study Group A. J Pediatr. 1974;84:131–4.
Sieber WK, Dibbins AW, Wiener ES. In paediatric surgery. 3d ed. In: Ravitch MM, Welch KJ, Benson CD, Aberdeen E, Randolph JG, editors. Yearbook Medical Publishers Inc; 1979. Ch. 99, p. 1095.
Evans AE, D’Angio GJ, Randolph J. A proposed staging system for children with neuroblastoma. Cancer. 1971;27:374–8.
Breslow N, McCann B. Statistical estimation of prognosis for children with neuroblastoma. Cancer Res. 1971;31:2098–103.
Brodeur GM, Seeger RC, Barrett A, et al. International criteria for diagnosis, staging and response to treatment in patients with neuroblastoma. J Clin Oncol. 1988;6:1874–81.
Brodeur GM, Pritchard J, Berthold F, et al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J Clin Oncol. 1993;11(8):1466–77.
Cohn SL, Pearson ADJ, London WB, et al. The International Neuroblastoma Risk Group classification system: an INRG task force report. J Clin Onc. 2009;27:289–97.
Monclair T, Brodeur GM, Ambros PF, et al. The international Neuroblastoma Risk Group (INRG) staging system: an INRG task force report. J Clin Oncol. 2009;27:298–303.
Cecchetto G, Mosseri V, Bernardi B, et al. Surgical risk factors in primary surgery for localised neuroblastoma: the LNESG1 study of the European International Society of Pediatric Oncology Neuroblastoma Group. J Clin Onc. 2005;23:8483–9.
Brisse HJ, McCarville MB, Granata C, Krug KB, Wootton-Gorges SL, Kanegawa K, … Monclair T. Guidelines for imaging and staging of neuroblastic tumors: consensus report from the International Neuroblastoma Risk Group Project. Radiology. 2011; 261:243–57.
Beckwith JB, Martin RF. Observations on the histopathology of neuroblastomas. J Ped Surg. 1968;3:106–10.
Shimada H, Chatten J, Newton WA, et al. Histopathologic prognostic factors in neuroblastic tumours: definitions of subtypes of ganglioneuroblastomaand an age linked classification of neuroblastomas. J NatCancer Inst. 1984;73:405–16.
Joshi VV, Cantor Altshuler G, et al. Age linked prognostic categorization based on a new histologic grading system of neuroblastomas. A clinicopathologic study of 211 cases from the Pediatric Oncology Group. Cancer. 1992;69:2197–211.
Shimada H, Ambros IM, Dehner LP, et al. Terminology and morphologic criteria of neuroblastic tumours: recommendations by the International Neuroblastoma Pathology Committee. Cancer. 1999;86:349–63.
Peuchmar M, d’Amore ES, Joshi VV, et al. Revision of the international neuroblastoma pathology classification: confirmation of the favourable and unfavourable diagnostic subsets in ganglioneuroblastoma, nodular. Cancer. 2003;98:2274–81.
Sano H, Bonadio J, Gerbing RB, et al. International neuroblastoma pathology classification adds independent prognostic information beyond the prognostic contribution of age. Eur J Cancer. 2006;42:1113–9.
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 Eng J Med. 1984;311:231–5.
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.
Brodeur GM, Sekhon G, Goldstein MN. Chromosomal aberrations in human neuroblastomas. Cancer. 1977;40:2256–63.
Spitz R, Hero B, Simon T, Berthold F. Loss in chromosome 11q identifies tumours with increased risk for metastatic relapses in localised and 4S neuroblastoma. Clin Cancer Res. 2006;12:3368–73.
Bown N, Cotteril S, Lastowska M, et al. Gain of chromosome arm 17q and adverse outcome in patients with neuroblastoma. N Eng J Med. 1999; 340:1954–61.
Chen Y, Takita J, Choi YL, et al. Oncogenic mutations of ALK kinase in neuroblastoma. Nature. 2008;455(7215):971–4.
Stiller CA. Trends in neuroblastoma in Great Britain: incidence and mortality 1971–1990. Eur J Cancer. 1993;29A:1008–12.
Gutierrez JC, Fischer AC, Sola JE, Perez CA, Koniaris LG. Markedly improving survival of neuroblastoma: a 30 year analysis of 1646 patients. Ped Surg Int. 2007;23:637–46.
Shimada H, Chatten J, Newton WA Jr, et al. Histopathologic prognostic factors in neuroblastic tumours: definition of subtypes of ganglioneuroblastoma and an age-linked classification of neuroblastomas. J Nat Cancer Inst. 1984;73:405–16.
Matthay KK, Perez C, Seeger RC, et al. Successful treatment of stage 3 neuroblastoma based on prospective biologic staging: a Children’s Cancer Group Study. J Clin Onc. 1998;16:1256–64.
Perez CA, Matthay KK, Atkinson JB, 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 Onc. 2000;18:18–26.
Brodeur GM. Neuroblastoma: biological insights into a clinical enigma. Nat Rev Cancer. 2003;3:203–16.
Maris JM, Hogarty MD, Bagatell R, et al. Neuroblastoma. Lancet. 2007; 369:2016–20.
O’Neill JA, Littman P, Blitzer P, Soper K, Chatten J, Shimada H. The role of surgery in localised neuroblastoma. J Ped Surg. 1985;20:708–12.
Matthay KK, Sather HN, Seeger RC, Haase GM, Hammond GD. Excellent outcome of stage II neuroblastoma is independent of residual disease and radiation therapy. J Clin Onc. 1989;7:236–44.
Evans AE, Silber JH, Shpilski A, D’Angio GJ. Successful management of low-stage Neuroblastoma without adjuvant therapies: a comparison of two decades, 1972 through 1981 and 1982 through 1992, in a single institution. J Clin Onc. 1996;14:2504–10.
Hero B, et al. Localised infant neuroblastomas often show spontaneous regression: results of the prospective trial NB95-S and NB97. J Clin Onc. 2008;26:1504–10.
Rubie H, Hartman O, Michon J, et al. N-Myc gene amplification is a major prognostic factor in localised neuroblastoma: results of the French neuroblastoma 90 study. J Clin Onc. 1997;15:1171–82.
Shamberger RC, Smith EI, Joshi W, et al. The risk of nephrectomy during local control in abdominal neuroblastoma. J Ped Surg. 1998;33:161–4.
Kiely E. A technique for excision of abdominal neuroblastoma. Ann RCS Eng. 2007;89:342–8.
LaQuaglia MP, Kushner BH, Heller G, Bonilla MA, Lindsley KL, Cheung N-KV. Stage 4 neuroblastoma diagnosed at more than 1 year of age: gross total resection and clinical outcome. J Ped Surg. 1994;29:1162–6.
Holmes K, et al. Advances in neuroblastoma research. Cologne: Plenary 12; 2014.
De Bernardi B, Mosseri V, Rubie H, et al. Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group. Br J Cancer. 2008;99:1027–33.
Kohler JA, Rubie H, Castel V, et al. Treatment of children over the age of one year with unresectable localised neuroblastoma without MYCN amplification: results of the SIOPEN study. Eur J Cancer. 2013;49(17):3671–9.
Haase GM, Wong KY, de Lorimer AA, Sather HN, Hammond GD. Improvement in survival after excision of primary tumor in stage III neuroblastoma. J Ped Surg. 1989;24:194–200.
Powis M, Imeson J, Holmes SJK. The effect of complete excision on stage III neuroblastoma: a report of the European Neuroblastoma Study Group. J Ped Surg. 1996;31:516–9.
Modak S, Kushner, LaQuaglia MP, Kramer K, Cheung NK. Management and outcome of stage 3 neuroblastoma. Eur J Cancer. 2009; 45:90–8.
Hero B, et al. Localised infant neuroblastomas often show spontaneous regression: results of the prospective trial NB95-S and NB97. J Clin Onc. 2008; 26(9):1504–10.
Park JR, Villablanca JG, London WB, et al. Outcome of high risk stage 3 neuroblastoma with myeloablative therapy and 13-cis-retinoic acid: A report from the Children’s Oncology Group. Ped Blood and Cancer. 2009;52:44–50.
Ladenstein R, Valteau-Couanet D, Brock P, et al. Randomized trial of prophylactic granulocyte colony-stimulating factor during rapid COJEC induction in pediatric patients with high-risk neuroblastoma: the European HR-NBL1/SIOPEN study. J Clin Onc. 2010; 28:3516–24.
Yu AL, Gilman, Ozkaynak MF, et al. Anti-GD2 antibody with GM-CSF, interleukin-2 and isotretinoin for neuroblastoma. NEng J Med. 2010; 363:1324–34.
Haase GM, O’Leary MC, Ramsay NK, et al. Aggressive surgery combined with intensive chemotherapy improves survival in poor risk neuroblastoma. J Ped Surg. 1991; 26:1119–23.
Tsuchida Y, Yokoyam J, Kaneko M, et al. Therapeutic significance of surgery in advanced neuroblastoma: a report from the Study Group of Japan. J Ped Surg. 1992; 27:616–22.
Rich BS, McEvoy MP, LaQuaglia MP, Wolden SL. Local control, survival and operative morbidity and mortality after re-resection and intra-operative radiation therapy for recurrent or persistent primary high-risk neuroblastoma. J Ped Surg. 2011;46:97–102.
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 Ped Surg. 2004;39:931–6.
Kaneko M, Ohakawa H, Iwakawa M. Is extensive surgery required for treatment of advanced neuroblastoma? J Ped Surg. 1997;32:1616–9.
Castel V, Tovar J, Costa J, et al. The role of surgery in stage IV neuroblastoma. J Ped Surg. 2002;37:1574–8.
Berthold F, Trechow R, Utsch S, Zieschang J. Prognostic factors in metastatic neuroblastoma. A multivariate analysis of 182 cases. Am J Ped Hemat Onc. 1992; 14:207–15.
Berthold F, Hero B, Kremens B, et al. Long-term results and risk profiles of patients in five consecutive trials (1979–1997) with stage 4 neuroblastoma over 1 year of age. Cancer Lett. 2003; 197:11–17.
Von Schweinitz D, Hero B, Berthold F. The impact of surgical radicality on outcome in childhood neuroblastoma. Eur J Surg. 2002;12:402–9.
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 Ped Surg. 2004; 39:412–7.
SimonT, Haberle B, Hero B, et al. Role of surgery in the treatment of patients with stage 4 neuroblastoma age 18 months or older at diagnosis. J Clin Oncol. 2013; 31:752–8.
Schleiermacher G, Rubie H, Hartmann O, Bergeron C, Chastagner P, Medinaud F, Michon J. Treatment of stage 4s neuroblastoma—report of 10 years experience of French Society of Paediatric Oncology (SFOP). B J Cancer. 2003;89:470–6.
Hsu LL, Evans AE, D’Angio GJ. Hepatomegaly in neuroblastoma stage 4S: criteria for treatment of the vulnerable neonate. Med Ped Onc. 1996; 27:521–8.
Simon T, Hero B, Bongartz R, Schmidt M, Muller RP, Berthold F. Intensified external-beam radiation therapy improves the outcome of stage 4 neuroblastoma in children >1 year with residual local disease. Strahlenther Onkol. 2006;182:389–94.
Haas-Kogan DA, Swift PS, Selch M, et al. Impact of radiotherapy for high risk neuroblastoma: a Children’s Cancer Group study. Int J Radiat Oncol Biol Phys. 2003;56:28–39.
Voute PA, Hoefnagel CA, De KJ, Valdes OR, Bakker DJ, van de Kleij AJ. Results of treatment with 131 I-meta-iodobenzylguanidine (131I MIBG) in patients with neuroblastoma. Future prospects of zetotherapy. Prog Clin Biol Res. 1991; 366:439–45.
Tuchman M, Morris CL, Ramnaraine ML, Bowers LD, Krivit W. Value of random urinary homovanillic and vanillylmandelic acid levels in the management of patients with neuroblastoma: comparison with 24 h urine collections. Pediatrics. 1985;75:234–8.
Sawada T. Past and future of neuroblastoma screening in Japan. Am J Pediatr Hematol Oncol. 1992;14:320–6.
Ajiki W, Tsukuma H, Oshima A, Kawa K. Effects of mass screening for neuroblastoma on incidence, mortality and survival rates in Osaka, Japan. Cancer Causes Control. 1998;9:631–6.
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Holmes, K. (2018). Neuroblastoma. In: Ledbetter, D., Johnson, P. (eds) Endocrine Surgery in Children. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-54256-9_9
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DOI: https://doi.org/10.1007/978-3-662-54256-9_9
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