Treatment and outcome of adult-onset neuroblastoma Journal Article


Authors: Suzuki, M.; Kushner, B. H.; Kramer, K.; Basu, E. M.; Roberts, S. S.; Hammond, W. J.; LaQuaglia, M. P.; Wolden, S. L.; Cheung, N. K. V.; Modak, S.
Article Title: Treatment and outcome of adult-onset neuroblastoma
Abstract: Adult-onset neuroblastoma is rare and little is known about its biology and clinical course. There is no established therapy for adult-onset neuroblastoma. Anti-GD2 immunotherapy is now standard therapy in children with high-risk neuroblastoma; however, its use has not been reported in adults. Forty-four adults (18–71 years old) diagnosed with neuroblastoma between 1979 and 2015 were treated at Memorial Sloan Kettering Cancer Center. Five, 1, 5 and 33 patients had INSS stage 1, 2, 3 and 4 diseases, respectively. Genetic abnormalities included somatic ATRX (58%) and ALK mutations (42%) but not MYCN-amplification. In the 11 patients with locoregional disease, 10-year progression-free (PFS) and overall survival (OS) was 35.4 ± 16.1% and 61.4 ± 15.3%, respectively. Among 33 adults with stage 4 neuroblastoma, 7 (21%) achieved complete response (CR) after induction chemotherapy and/or surgery. Seven patients with primary refractory neuroblastoma (all with osteomedullary but no soft tissue disease) received anti-GD2 antibodies, mouse or humanized 3F8. Antibody-related adverse events were similar to those in children, response rate being 71.4%. In patients with stage 4 disease at diagnosis, 5-year PFS was 9.7± 5.3% and most patients who were alive with disease at 5 years died of neuroblastoma over the next 5 years, 10-year OS being only 19.0 ± 8.2%. Patients who achieved CR after induction had superior PFS and OS (p = 0.006, p = 0.031, respectively). Adult-onset neuroblastoma appeared to have different biology from pediatric or adolescent NB, and poorer outcome. Complete disease control appeared to improve long-term survival. Anti-GD2 immunotherapy was well tolerated and might be beneficial. © 2018 UICC
Keywords: adult; cancer survival; clinical article; controlled study; human tissue; treatment response; aged; cancer surgery; primary tumor; survival rate; unclassified drug; overall survival; somatic mutation; drug tolerability; cancer localization; cisplatin; doxorubicin; cancer combination chemotherapy; drug efficacy; cancer staging; antineoplastic agent; cancer diagnosis; cancer immunotherapy; progression free survival; gene amplification; etoposide; cyclophosphamide; vincristine; retrospective study; cancer mortality; monoclonal antibody; survival time; cancer center; neuroblastoma; genetic disorder; cancer control; onset age; induction chemotherapy; bone marrow disease; anaplastic lymphoma kinase; clinical outcome; mycn gene; alk gene; atrx gene; monoclonal antibody gd2; human; male; female; priority journal; article; transcriptional regulator atrx; anti-gd2 immunotherapy
Journal Title: International Journal of Cancer
Volume: 143
Issue: 5
ISSN: 0020-7136
Publisher: John Wiley & Sons  
Date Published: 2018-09-01
Start Page: 1249
End Page: 1258
Language: English
DOI: 10.1002/ijc.31399
PROVIDER: scopus
PMCID: PMC6103828
PUBMED: 29574715
DOI/URL:
Notes: Article -- Export Date: 4 September 2018 -- Source: Scopus
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MSK Authors
  1. Brian Kushner
    190 Kushner
  2. Nai-Kong Cheung
    439 Cheung
  3. Kim Kramer
    168 Kramer
  4. Shakeel Modak
    159 Modak
  5. Suzanne L Wolden
    419 Wolden
  6. Ellen Marlese Basu
    34 Basu
  7. Maya Suzuki
    9 Suzuki
  8. Stephen Stacy Roberts
    40 Roberts