BACKGROUND: We previously reported that stage 3 neuroblastoma comprises (i) a low-risk group including all infants (age 0-11 months) as well as older children with non-abdominal primaries, and (ii) a high-risk group made up of children >1 year of age with abdominal primaries. Aggressive chemotherapy was effective only in the latter group. PATIENTS AND TREATMENT: On this basis, in 1990 we designed a new protocol by which all low-risk patients received standard-dose chemotherapy, while the high-risk ones received very aggressive chemotherapy. RESULTS: Between November 1990 and December 1997 a total of 95 eligible and evaluable children were enrolled: 47 were low-risk (35 infants and 12>1 year of age at diagnosis and having non-abdominal primaries), and 48 were high-risk (being >1 year of age and having abdominal primaries). Of the 47 low-risk patients, five relapsed and four subsequently died. The 5-year overall survival (OS) was 91%. Of the 48 patients in the high-risk group, 22 relapsed or progressed, 18 of whom died from their disease and two from toxicity, and one was lost to follow-up. The 5-year OS was 60%. Univariate analysis showed that age, site of primary, risk-group, urine vanillylmandelic excretion, plasma levels of lactate dehydrogenase, ferritin and neurone-specific enolase, and MYCN status correlated with outcome. However, multivariate analysis showed that only MYCN status retained prognostic value. CONCLUSIONS: In low-risk stage 3 neuroblastoma, standard-dose chemotherapy is associated with an excellent chance of being cured. Aggressive chemotherapy is effective for high-risk patients, but results are still unsatisfactory. MYCN gene amplification is a prognostic indicator for most, but not all, treatment failures.
BACKGROUND: We previously reported that stage 3 neuroblastoma comprises (i) a low-risk group including all infants (age 0-11 months) as well as older children with non-abdominal primaries, and (ii) a high-risk group made up of children >1 year of age with abdominal primaries. Aggressive chemotherapy was effective only in the latter group. PATIENTS AND TREATMENT: On this basis, in 1990 we designed a new protocol by which all low-risk patients received standard-dose chemotherapy, while the high-risk ones received very aggressive chemotherapy. RESULTS: Between November 1990 and December 1997 a total of 95 eligible and evaluable children were enrolled: 47 were low-risk (35 infants and 12>1 year of age at diagnosis and having non-abdominal primaries), and 48 were high-risk (being >1 year of age and having abdominal primaries). Of the 47 low-risk patients, five relapsed and four subsequently died. The 5-year overall survival (OS) was 91%. Of the 48 patients in the high-risk group, 22 relapsed or progressed, 18 of whom died from their disease and two from toxicity, and one was lost to follow-up. The 5-year OS was 60%. Univariate analysis showed that age, site of primary, risk-group, urine vanillylmandelic excretion, plasma levels of lactate dehydrogenase, ferritin and neurone-specific enolase, and MYCN status correlated with outcome. However, multivariate analysis showed that only MYCN status retained prognostic value. CONCLUSIONS: In low-risk stage 3 neuroblastoma, standard-dose chemotherapy is associated with an excellent chance of being cured. Aggressive chemotherapy is effective for high-risk patients, but results are still unsatisfactory. MYCN gene amplification is a prognostic indicator for most, but not all, treatment failures.
Authors: Thomas E Ichim; Boris Minev; Todd Braciak; Brandon Luna; Ron Hunninghake; Nina A Mikirova; James A Jackson; Michael J Gonzalez; Jorge R Miranda-Massari; Doru T Alexandrescu; Constantin A Dasanu; Vladimir Bogin; Janis Ancans; R Brian Stevens; Boris Markosian; James Koropatnick; Chien-Shing Chen; Neil H Riordan Journal: J Transl Med Date: 2011-03-04 Impact factor: 5.531
Authors: Shakeel Modak; Brian H Kushner; Michael P LaQuaglia; Kim Kramer; Nai-Kong V Cheung Journal: Eur J Cancer Date: 2008-11-06 Impact factor: 9.162
Authors: Patrick J Bastian; Gudrun Fleischhack; Martina Zimmermann; Carola Hasan; Udo Bode; Stefan C Müller; Stefan Schumacher Journal: World J Urol Date: 2004-09-14 Impact factor: 4.226
Authors: Brian H Kushner; Michael P LaQuaglia; Shakeel Modak; Suzanne L Wolden; Ellen M Basu; Stephen S Roberts; Kim Kramer; Karima Yataghene; Irene Y Cheung; Nai-Kong V Cheung Journal: Oncotarget Date: 2017-08-24
Authors: Giuliana Cangemi; Giorgio Reggiardo; Sebastiano Barco; Laura Barbagallo; Massimo Conte; Paolo D'Angelo; Maurizio Bianchi; Claudio Favre; Barbara Galleni; Giovanni Melioli; Riccardo Haupt; Alberto Garaventa; Maria V Corrias Journal: Onco Targets Ther Date: 2012-11-30 Impact factor: 4.147
Authors: B De Bernardi; V Mosseri; H Rubie; V Castel; A Foot; R Ladenstein; G Laureys; M Beck-Popovic; A F de Lacerda; A D J Pearson; J De Kraker; P F Ambros; Y de Rycke; M Conte; P Bruzzi; J Michon Journal: Br J Cancer Date: 2008-09-02 Impact factor: 7.640