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There is no established therapy for adult\u2010onset neuroblastoma. Anti\u2010GD2 immunotherapy is now standard therapy in children with high\u2010risk neuroblastoma; however, its use has not been reported in adults. Forty\u2010four adults (18\u201371 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 <jats:italic>ATRX<\/jats:italic> (58%) and <jats:italic>ALK<\/jats:italic> mutations (42%) but not <jats:italic>MYCN<\/jats:italic>\u2010amplification. In the 11 patients with locoregional disease, 10\u2010year progression\u2010free (PFS) and overall survival (OS) was 35.4\u2009\u00b1\u200916.1% and 61.4\u2009\u00b1\u200915.3%, 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\u2010GD2 antibodies, mouse or humanized 3F8. Antibody\u2010related adverse events were similar to those in children, response rate being 71.4%. In patients with stage 4 disease at diagnosis, 5\u2010year PFS was 9.7\u00b1 5.3% and most patients who were alive with disease at 5 years died of neuroblastoma over the next 5 years, 10\u2010year OS being only 19.0\u2009\u00b1\u20098.2%. Patients who achieved CR after induction had superior PFS and OS (<jats:italic>p<\/jats:italic>\u2009=\u20090.006, <jats:italic>p<\/jats:italic>\u2009=\u20090.031, respectively). Adult\u2010onset neuroblastoma appeared to have different biology from pediatric or adolescent NB, and poorer outcome. Complete disease control appeared to improve long\u2010term survival. Anti\u2010GD2 immunotherapy was well tolerated and might be beneficial.<\/jats:p>","DOI":"10.1002\/ijc.31399","type":"journal-article","created":{"date-parts":[[2018,3,25]],"date-time":"2018-03-25T15:07:12Z","timestamp":1521990432000},"page":"1249-1258","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Treatment and outcome of adult\u2010onset neuroblastoma"],"prefix":"10.1002","volume":"143","author":[{"given":"Maya","family":"Suzuki","sequence":"first","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian H.","family":"Kushner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim","family":"Kramer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen M.","family":"Basu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen S.","family":"Roberts","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William J.","family":"Hammond","sequence":"additional","affiliation":[{"name":"Department of Surgery Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"LaQuaglia","sequence":"additional","affiliation":[{"name":"Department of Surgery Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne L.","family":"Wolden","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nai\u2010Kong V.","family":"Cheung","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shakeel","family":"Modak","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center New York NY"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,4,6]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0804577"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.05.571"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20859"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/2162-3619-3-20"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24777"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1155\/2014\/375151"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.10273"},{"key":"e_1_2_11_9_1","first-page":"1434","article-title":"Neuroblastoma in adolescence and adulthood","volume":"75","author":"Tang CK","year":"1975","journal-title":"N Y State J Med"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197603)37:3<1334::AID-CNCR2820370314>3.0.CO;2-Y"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197801)41:1<56::AID-CNCR2820410110>3.0.CO;2-2"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.55.646.579"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19800301)45:5<840::AID-CNCR2820450503>3.0.CO;2-Z"},{"key":"e_1_2_11_14_1","first-page":"352","article-title":"Neuroblastoma in adults. 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