PURPOSE: MYCN amplification is rarely detected in patients with favorable-stage neuroblastoma (NB). To determine the clinical significance of MYCN amplification in children with favorable-stage NB, we performed a retrospective review of data from the Pediatric Oncology Group (POG) biology study 9047. PATIENTS AND METHODS: MYCN status, tumor cell ploidy, treatment, and outcome of patients with stage A, B, or Ds NB, enrolled on POG 9047 between 1990 and 1999 were analyzed. Event-free survival (EFS) and overall (OS) survival rates were analyzed using the Kaplan-Meier method. RESULTS: Of the 1,667 patients enrolled on POG 9047, 643 had favorable-stage disease. Of these, follow-up data were available on 568 (34%) with stage A, B, or Ds disease and normal MYCN copy number, and 32 (1.9%) patients with MYCN-amplified, stage A, B, or Ds tumors. Within the cohort lacking MYCN amplification, the 7-year EFS and OS rates (+/- SE) were 91% +/- 1% and 96% +/- 1%, respectively. Patients with MYCN amplification had significantly worse EFS and OS (50% +/- 9% and 59% +/- 9%, respectively, P < .0001). Within the cohort of children with MYCN amplification, the 7-year EFS and OS rates were 80% +/- 10% and 87% +/- 9%, respectively for patients with hyperdiploid tumors and 25% +/- 11% and 38% +/- 12% for patients with diploid/hypodiploid NBs (P = .0063 and P = .0074, respectively). CONCLUSION: Tumor cell ploidy may be a clinically useful factor for prognostication and treatment stratification in children with MYCN-amplified, favorable-stage NB tumors.
PURPOSE:MYCN amplification is rarely detected in patients with favorable-stage neuroblastoma (NB). To determine the clinical significance of MYCN amplification in children with favorable-stage NB, we performed a retrospective review of data from the Pediatric Oncology Group (POG) biology study 9047. PATIENTS AND METHODS: MYCN status, tumor cell ploidy, treatment, and outcome of patients with stage A, B, or Ds NB, enrolled on POG 9047 between 1990 and 1999 were analyzed. Event-free survival (EFS) and overall (OS) survival rates were analyzed using the Kaplan-Meier method. RESULTS: Of the 1,667 patients enrolled on POG 9047, 643 had favorable-stage disease. Of these, follow-up data were available on 568 (34%) with stage A, B, or Ds disease and normal MYCN copy number, and 32 (1.9%) patients with MYCN-amplified, stage A, B, or Ds tumors. Within the cohort lacking MYCN amplification, the 7-year EFS and OS rates (+/- SE) were 91% +/- 1% and 96% +/- 1%, respectively. Patients with MYCN amplification had significantly worse EFS and OS (50% +/- 9% and 59% +/- 9%, respectively, P < .0001). Within the cohort of children with MYCN amplification, the 7-year EFS and OS rates were 80% +/- 10% and 87% +/- 9%, respectively for patients with hyperdiploid tumors and 25% +/- 11% and 38% +/- 12% for patients with diploid/hypodiploid NBs (P = .0063 and P = .0074, respectively). CONCLUSION:Tumor cell ploidy may be a clinically useful factor for prognostication and treatment stratification in children with MYCN-amplified, favorable-stage NB tumors.
Authors: Denah R Taggart; Wendy B London; Mary Lou Schmidt; Steven G DuBois; Tom F Monclair; Akira Nakagawara; Bruno De Bernardi; Peter F Ambros; Andrew D J Pearson; Susan L Cohn; Katherine K Matthay Journal: J Clin Oncol Date: 2011-10-03 Impact factor: 44.544
Authors: Elizabeth Sokol; Ami V Desai; Mark A Applebaum; Dominique Valteau-Couanet; Julie R Park; Andrew D J Pearson; Gudrun Schleiermacher; Meredith S Irwin; Michael Hogarty; Arlene Naranjo; Samuel Volchenboum; Susan L Cohn; Wendy B London Journal: J Clin Oncol Date: 2020-04-21 Impact factor: 44.544
Authors: Rochelle Bagatell; Maja Beck-Popovic; Wendy B London; Yang Zhang; Andrew D J Pearson; Katherine K Matthay; Tom Monclair; Peter F Ambros; Susan L Cohn Journal: J Clin Oncol Date: 2008-12-01 Impact factor: 44.544
Authors: Wendy B London; Rochelle Bagatell; Brenda J Weigel; Elizabeth Fox; Dongjing Guo; Collin Van Ryn; Arlene Naranjo; Julie R Park Journal: Cancer Date: 2017-09-08 Impact factor: 6.860