title: [Occurrence and prognostic impact of selected factors in neuroblastoma in children].
abstract: Neuroblastoma (NBL) accounts for 6-10% of neoplastic diseases of childhood. The clinical course of NBL is very variable and depends on the presence of prognostic factors. The aim of the study is retrospective evaluation of occurrence of known prognostic factors and disease markers in patients treated in 1991-2005 in our institution. Sixty of 100 treated patients were included to the study, in whom tumor embedded in paraffin was available. In all patients, who had no genetic evaluation at diagnosis, especially MYCN amplification, FISH study was performed. In analyzed group, 29 patients died, 23 of them from NBL. Disease progression (n = 12) or relapse (n = 19) was observed in 31 patients. In the whole analyzed group, age had statistically significant influence on deaths caused by NBL (p = 0.01) and therapy failures (p = 0.00008). The statistically significant increase of NBL death incidence (p = 0.00001) and therapy failures (p = 0.00004) was found in stage 4 patients in comparison with other stages. Presence of MYCN amplification statistically significant decreases overall survival (OS) (p = 0.01) and disease free survival (DFS) (p = 0.047) for the whole analyzed group. However, presence of MYCN amplification had no statistically significant influence on OS and DFS in patients over 1 year of age. Multiple Cox analysis showed independent statistically significant influence of stage and MYCN amplification on prognosis. Employment of new treatment modalities, with treatment intensity adjusted to the risk group, but also with the specificity adapted to tumor characteristics (cytogenetic and molecular) as well as further development of supportive therapy improvement may lead to improvement of treatment results, especially in high risk group and to reduction of therapy complications and improvement of quality of life in all children with this tumor.
authors.affiliation: Klinika Onkologii i Hematologii Dzieciecej, Polsko-Amerykański Instytut Pediatrii, UJ CM, Kraków.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Wieczorek
authors.forename: Walentyna
authors.initials: W
authors.lastname: Balwierz
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005784:Gene Amplification; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D000071447:N-Myc Proto-Oncogene Protein; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D009687:Nuclear Proteins / Q000235:genetics; D015513:Oncogene Proteins / Q000235:genetics; D011044:Poland / Q000453:epidemiology; D011379:Prognosis; D011788:Quality of Life; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins