PMID- 20080471 OWN - NLM STAT- MEDLINE DCOM- 20100402 LR - 20211020 IS - 1699-3055 (Electronic) IS - 1699-048X (Linking) VI - 12 IP - 1 DP - 2010 Jan TI - Neuroblastoma in adolescents: genetic and clinical characterisation. PG - 49-54 LID - 10.1007/s12094-010-0466-z [doi] AB - BACKGROUND: Age at diagnosis is an important risk factor in neuroblastoma (NB) with worse prognosis in children older than 18 months. A more indolent course with long-term relapses and fatal outcome has been described in small series of adolescents. Our objective was to describe biological factors that contribute to this particular behaviour and could be helpful in their treatment. PROCEDURE: NB cases older than 10 years of age at diagnosis registered in the files of the Neuroblastoma Group of SEOP from 1992 to 2007 were included. Disease extension was classified according to the International Neuroblastoma Staging System (INSS). Tumour samples were studied according to the International Neuroblastoma Pathology Classification (INPC). Biological studies included MNA, 1p, 11q and 17q status and ploidy. RESULTS: Twenty-two patients, from 10.1 to 24.6 years old, were included. Advanced stages predominated. 14/17 patients presented unfavourable histology. None had NMA or 1p del. However, 11q del was found in 8/13 cases and 17q gain in 7/11. Overall survival (OS) and event-free survival (EFS) for the entire series at 5 years were 0.45 and 0.32, respectively. Moreover, 5-year OS and EFS for stage 4 patients were 0.33 and 0.15. CONCLUSIONS: NB in adolescents is a special subgroup characterised by high-risk prognostic features which differ from those seen in younger patients, especially in relation to genetic abnormalities. The outcome in stage 4 was worse than in younger metastatic children, outlining the need for new therapeutic approaches in this subgroup of patients. The exact cut-off to separate older patients has not yet been established and will probably be based on biology. FAU - Castel, Victoria AU - Castel V AD - Unidad de Oncologia Pediatrica, Hospital Universitario La Fe, Valencia, Spain. castel_vic@gva.es FAU - Villamon, Eva AU - Villamon E FAU - Canete, Adela AU - Canete A FAU - Navarro, Samuel AU - Navarro S FAU - Ruiz, Amparo AU - Ruiz A FAU - Melero, Carmen AU - Melero C FAU - Herrero, Antonio AU - Herrero A FAU - Yanez, Yania AU - Yanez Y FAU - Noguera, Rosa AU - Noguera R LA - eng PT - Journal Article PL - Italy TA - Clin Transl Oncol JT - Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico JID - 101247119 RN - 0 (MYCN protein, human) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (Nuclear Proteins) RN - 0 (Oncogene Proteins) SB - IM MH - Abdominal Neoplasms/*diagnosis/epidemiology/genetics/pathology MH - Adolescent MH - Adult MH - Age of Onset MH - Child MH - Chromosome Aberrations/statistics & numerical data MH - Chromosomes, Human, Pair 1 MH - Chromosomes, Human, Pair 11 MH - Female MH - Humans MH - In Situ Hybridization, Fluorescence MH - Male MH - N-Myc Proto-Oncogene Protein MH - Neoplasm Metastasis MH - Neuroblastoma/*diagnosis/epidemiology/genetics/pathology MH - Nuclear Proteins/genetics MH - Oncogene Proteins/genetics MH - Prognosis MH - Recurrence MH - Retrospective Studies MH - Young Adult EDAT- 2010/01/19 06:00 MHDA- 2010/04/03 06:00 CRDT- 2010/01/19 06:00 PHST- 2010/01/19 06:00 [entrez] PHST- 2010/01/19 06:00 [pubmed] PHST- 2010/04/03 06:00 [medline] AID - CLAT301 [pii] AID - 10.1007/s12094-010-0466-z [doi] PST - ppublish SO - Clin Transl Oncol. 2010 Jan;12(1):49-54. doi: 10.1007/s12094-010-0466-z.