10 years' neuroblastoma screening in Europe: preliminary results of a clinical and biological review from the Study Group for Evaluation of Neuroblastoma Screening in Europe (SENSE).
Between January 1986 and May 1996, 870,313 children were tested in European neuroblastoma (NB) screening programmes. Among these children, 82 cases of NB (age range 4-24 months, median 11 months) were detected by screening. 83% of the patients had localised NB and 17% were diagnosed with generalised NB (stage 4, 10%; stage 4s, 7%). Unfavourable biological markers (MYCN amplification, loss of heterozygosity (LOH) 1p36, DNA di/tetraploidy) were observed in 14% of 76 biologically examined cases. The median follow-up time of all the patients was 21.5 months (range 1-101 months). To date, 69 patients are in complete remission (CR) and 2 patients have died due to therapy (stage 4, 1 patient; stage 3, 1 patient with unfavourable markers). Apart from screened patients, 16 other patients with NB were found who had previously had a normal screening test, i.e. 'false negative' patients (age range 10-41 months, median 31.5 months). The median interval between screening and diagnosis was 24.5 months (range 6-35 months). 11 of the 'false negative' patients suffered from generalised NB (stage 4) and 5 had localised NB at diagnosis. Unfavourable biological markers were observed in 7/12 patients. 5 patients have died, 2 achieved partial remission and 9 CR. 9 of the 11 patients with unfavourable biological markers diagnosed due to NB screening are currently in CR. It is very likely that, among the patients without unfavourable biological markers, we detected tumours which may have regressed spontaneously. These children may have undergone 'unnecessary,' but unavoidable, diagnostic procedures and therapy. To reduce the number of 'false negative' patients, a later screening could be helpful and should be evaluated.
Erttmann
R
R
Department of Pediatric Hematology, Universitätskinderklinik, Hamburg, Germany.
Tafese
T
T
Berthold
F
F
Kerbl
R
R
Mann
J
J
Parker
L
L
Schilling
F
F
Ambros
P
P
Christiansen
H
H
Favrot
M
M
Kabisch
H
H
Hero
B
B
Philip
T
T
D017677:Age Distribution; D014408:Biomarkers, Tumor; D002675:Child, Preschool; D005060:Europe; D016259:Genes, myc; D006801:Humans; D007223:Infant; D019656:Loss of Heterozygosity; D008403:Mass Screening / Q000592:standards*; D009447:Neuroblastoma / Q000235:genetics / Q000517:prevention & control*; D011003:Ploidies; D011379:Prognosis; D012307:Risk Factors; D012680:Sensitivity and Specificity; D017678:Sex Distribution; D016019:Survival Analysis
D014408:Biomarkers, Tumor