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. 2018 Sep 1;143(5):1249-1258.
doi: 10.1002/ijc.31399. Epub 2018 Apr 6.

Treatment and outcome of adult-onset neuroblastoma

Maya Suzuki  1 , Brian H Kushner  1 , Kim Kramer  1 , Ellen M Basu  1 , Stephen S Roberts  1 , William J Hammond  2 , Michael P LaQuaglia  2 , Suzanne L Wolden  3 , Nai-Kong V Cheung  1 , Shakeel Modak  1
Affiliations

Treatment and outcome of adult-onset neuroblastoma

Maya Suzuki et al. Int J Cancer. .

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.

Keywords: adult; anti-GD2 immunotherapy; neuroblastoma.

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Figures

Figure 1
Figure 1
Patients with locoregional NB had superior PFS and OS than those who had metastatic disease at diagnosis in univariate analysis.
Figure 2
Figure 2
Achieving CR after induction and/or surgery was associated with favorable PFS and OS in adults with stage 4 NB.
See this image and copyright information in PMC

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