title: Long-term results and risk profiles of patients in five consecutive trials (1979-1997) with stage 4 neuroblastoma over 1 year of age.
abstract: During the last two decades new diagnostic and therapeutic tools have been utilized to improve the poor survival chances of children with stage 4 neuroblastoma. This study reviews the risk profiles and the long-term outcome of patients from five consecutive German neuroblastoma trials. A total of 96% of all German patients registered at the German childhood cancer registry with neuroblastoma stage 4 over 1 year of age at diagnosis entered one of the trials during 1979-2001. Eight hundred and twenty-eight consecutive children were analyzed retrospectively. In spite of having significantly improved diagnostic tools like bone marrow superstaging and mIBG scintigraphy the stage 4 incidence did not increase after reaching completeness of the registry (5.4 cases/100,000 children at 1-14 years of age; P=0.52). The distribution of the primary tumors and of metastases was constant over the periods. The amount of bone marrow infiltration did not change with time. The risk factors lactate dehydrogenase, ferritin and MYCN, and the clinical risk groups 4A, 4B, 4C also remained constant over the trials with a few exceptions for NB97. The 5-year event free survival increased from 0.01+/-0.01 (NB79) to 0.14+/-0.03 (NB85), 0.16+/-0.04 (NB82), 0.27+/-0.02 (NB90), and 0.33+/-0.04 (NB97). The overall survival rates improved similarly from 0.04 (NB79) to 0.44 (NB97). In conclusion, the improved survival was associated with better treatment and not caused by lower risk profiles in stage 4 neuroblastoma patients.
authors.affiliation: Department of Pediatric Oncology and Hematology, Children's Hospital, University of Cologne, Joseph-Stelzmann-Str. 9, 50924 Cologne, Germany. frank.berthold@medizin.uni-koeln.de
authors.forename: F
authors.initials: F
authors.lastname: Berthold
authors.forename: B
authors.initials: B
authors.lastname: Hero
authors.forename: B
authors.initials: B
authors.lastname: Kremens
authors.forename: R
authors.initials: R
authors.lastname: Handgretinger
authors.forename: G
authors.initials: G
authors.lastname: Henze
authors.forename: F H
authors.initials: FH
authors.lastname: Schilling
authors.forename: M
authors.initials: M
authors.lastname: Schrappe
authors.forename: T
authors.initials: T
authors.lastname: Simon
authors.forename: C
authors.initials: C
authors.lastname: Spix
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D005293:Ferritins / Q000378:metabolism; D016259:Genes, myc / Q000502:physiology; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D005293:Ferritins; D007770:L-Lactate Dehydrogenase