PMID- 23907002 OWN - NLM STAT- MEDLINE DCOM- 20140120 LR - 20220321 IS - 1879-0852 (Electronic) IS - 0959-8049 (Linking) VI - 49 IP - 17 DP - 2013 Nov TI - Treatment of children over the age of one year with unresectable localised neuroblastoma without MYCN amplification: results of the SIOPEN study. PG - 3671-9 LID - S0959-8049(13)00546-7 [pii] LID - 10.1016/j.ejca.2013.07.002 [doi] AB - BACKGROUND: In children older than 1 year with localised unresectable neuroblastoma (NB), treatment strategies are heterogeneous according to the national groups. The objective of this phase III non-randomised study was to evaluate the efficacy of conventional chemotherapy followed by surgery. PATIENTS AND METHODS: In the presence of surgical risk factors (SRF), six courses of chemotherapy alternating Carboplatin-Etoposide and Vincristin-Cyclophosphamide-Doxorubicin were given, and surgical resection was attempted after four. Survival analyses were performed using an intention-to-treat approach. The main objective was to achieve a 5-year survival over 80%. RESULTS: Out of 191 registered children, 160 were evaluable. There were 62.5% older than 18 months and 52.5% had unfavourable histology according to International Neuroblastoma Pathology Classification (INPC). Chemotherapy reduced the number of SRFs by one third. Delayed surgery was attempted in 86.3% of patients and was complete or nearly complete in 74%. The 5-year EFS and OS were 76.4% and 87.6% respectively, with significant better results for patients younger than 18 months or with favourable histology. CONCLUSION: This strategy provides encouraging results in children older than 1 year or 12 months with localised unresectable NB without MYCN amplification. However, in children older than 18 months and with unfavourable histology, additional treatment is recommended. CI - Copyright (c) 2013 Elsevier Ltd. All rights reserved. FAU - Kohler, J A AU - Kohler JA AD - Department of Paediatric Oncology, Southampton General Hospital, Southampton, UK. FAU - Rubie, H AU - Rubie H FAU - Castel, V AU - Castel V FAU - Beiske, K AU - Beiske K FAU - Holmes, K AU - Holmes K FAU - Gambini, C AU - Gambini C FAU - Casale, F AU - Casale F FAU - Munzer, C AU - Munzer C FAU - Erminio, G AU - Erminio G FAU - Parodi, S AU - Parodi S FAU - Navarro, S AU - Navarro S FAU - Marquez, C AU - Marquez C FAU - Peuchmaur, M AU - Peuchmaur M FAU - Cullinane, C AU - Cullinane C FAU - Brock, P AU - Brock P FAU - Valteau-Couanet, D AU - Valteau-Couanet D FAU - Garaventa, A AU - Garaventa A FAU - Haupt, R AU - Haupt R LA - eng PT - Clinical Trial, Phase III PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20130729 PL - England TA - Eur J Cancer JT - European journal of cancer (Oxford, England : 1990) JID - 9005373 RN - 0 (MYCN protein, human) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (Nuclear Proteins) RN - 0 (Oncogene Proteins) RN - 5J49Q6B70F (Vincristine) RN - 6PLQ3CP4P3 (Etoposide) RN - 80168379AG (Doxorubicin) RN - 8N3DW7272P (Cyclophosphamide) RN - BG3F62OND5 (Carboplatin) SB - IM MH - Adolescent MH - Age Factors MH - Antineoplastic Combined Chemotherapy Protocols/*therapeutic use MH - Carboplatin/administration & dosage MH - Child MH - Child, Preschool MH - Combined Modality Therapy MH - Cyclophosphamide/administration & dosage MH - Doxorubicin/administration & dosage MH - Etoposide/administration & dosage MH - Female MH - *Gene Amplification MH - Humans MH - Infant MH - Male MH - N-Myc Proto-Oncogene Protein MH - Neuroblastoma/*drug therapy/genetics/mortality MH - Nuclear Proteins/*genetics MH - Oncogene Proteins/*genetics MH - Survival Analysis MH - Vincristine/administration & dosage OTO - NOTNLM OT - Children OT - Conventional chemotherapy OT - Localised neuroblastoma OT - Unresectable EDAT- 2013/08/03 06:00 MHDA- 2014/01/21 06:00 CRDT- 2013/08/03 06:00 PHST- 2013/01/18 00:00 [received] PHST- 2013/06/06 00:00 [revised] PHST- 2013/07/01 00:00 [accepted] PHST- 2013/08/03 06:00 [entrez] PHST- 2013/08/03 06:00 [pubmed] PHST- 2014/01/21 06:00 [medline] AID - S0959-8049(13)00546-7 [pii] AID - 10.1016/j.ejca.2013.07.002 [doi] PST - ppublish SO - Eur J Cancer. 2013 Nov;49(17):3671-9. doi: 10.1016/j.ejca.2013.07.002. Epub 2013 Jul 29.