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Purpose: A significant increase in the incidence of neuroblastoma occurred among a 5-year birth cohort (May 1989 to April 1994) during an active urinary screening program for its early detection. We examined the postscreening incidence of neuroblastoma in the subsequent 5-year birth cohort (May 1994 to April 1999), with follow-up to 2002, to determine whether the incidence remained increased., Patients and Methods: We reviewed institutional records of patients diagnosed with neuroblastoma during the period from 1994 to 2002 who were born in 1994 to 1999 in the province of Quebec, as well as in the state of Minnesota and the province of Ontario, regions that had served as controls during the screening interval. We calculated and compared incidence rates during the 1994 to 2002 time period., Results: For the 5-year birth cohort as a whole, the rate of newly diagnosed neuroblastoma was higher in Quebec than in the control populations of Minnesota and Ontario (standardized incidence ratio, 1.34; 95% CI, 1.03 to 1.70). However, in years 4 and 5 of the interval, population-based incidence declined to the same levels as those seen in the control areas., Conclusion: The institution of a urinary screening program for neuroblastoma led to increased awareness of the diagnosis and an elevated rate of diagnosis even after the completion of the screening evaluation. However, this halo effect was transient, with diagnostic rates subsequently decreasing within the range seen in control populations.

Background: Twin concordance studies help evaluate the contribution of genetic factors in childhood cancers, but previous reports have primarily focused on leukemia because of the rarity of other malignancies. In the current report, a large cohort of childhood cancer survivors was used to: (1) describe twin concordance patterns for a range of cancers, (2) calculate the standardized incidence rates of cancers in twins, and (3) describe clinical features and outcomes of concordant twins., Procedure: Cancer family history was obtained on the 211 twins participating in the Childhood Cancer Survivor Study (CCSS) (14,352 participants surviving > or =5 years after a malignancy diagnosed at <21 years during January 1, 1970-December 31, 1986) to calculate probandwise twin concordance rates and standardized incidence ratios (SIRs) using Surveillance, Epidemiology, and End-Results data., Results: Seven monozygotic twin pairs were concordant for cancer (six for leukemia, one for non-Hodgkin lymphoma), yielding probandwise concordance rates of 9.5%, 20.7%, and 20.0% for all cancer, leukemia, and non-Hodgkin lymphoma (NHL), respectively. No concordance was observed among dizygotic twins or for dissimilar cancers. The SIR in monozygotic twins was 23.3 (95% CI = 11.1-48.9) for all cancer, 112.4 (95% CI = 50.5-250.1) for leukemia, and 40.5 (5.7-287.5) for NHL. Concordant twins were similar in age at diagnosis and vital status., Conclusions: Twin concordance for cancer is largely restricted to monozygotic twins and hematological malignancies, consistent with in utero malignancy transmission demonstrated by others. Our data support clinical monitoring of the twins of cases with hematological malignancies, and does not contribute evidence for genetic factors in other cancers.

Background: Well-designed evaluations of health services are frequently made today. However, the extent of the evaluations' benefits and costs is not well documented, creating uncertainty whether their use is optimal from society's perspective. We examined these costs and benefits using data from one well-designed evaluation, the Quebec Neuroblastoma Screening Project (QNSP). It screened most Quebec newborns between 1989 and 1994 for neuroblastoma. As previously reported, the screening did not reduce neuroblastoma mortality and caused adverse health effects., Methods: We compared the cost of doing the QNSP with its benefits. Had the QNSP not been undertaken, neuroblastoma screening would have been implemented throughout North America. We assume that screening would have started in 1989 and ended in 2002. The QNSP's benefits include the health costs and adverse health effects averted by not using ineffective screening during those 14 years. In our calculations we used neuroblastoma incidence data for the QNSP and for Ontario where there was no screening, detailed data describing the health services used by the patients, and Quebec cost data for those services., Results: The QNSP cost 8.77 million dollars (2002 US dollars). By not implementing similar screening programs between 1989 and 2002, the United States and Canada avoided 574.1 million dollars in health costs, the unnecessary treatment of 9223 children, and false-positive findings for 5003 children screened., Conclusions: The health care costs and adverse health effects averted by the QNSP justify its costs. These results show that well-designed evaluations can yield--at least sometimes--benefits substantially greater than their high costs. This raises an important policy issue: are these evaluations now being under- or over used?

Background: With increasing numbers of childhood cancer survivors, direct sequelae of cancer therapy and psychosocial outcomes are becoming more important. The authors described psychosocial outcomes (education, employment, health insurance, and marriage) for survivors of pediatric lower extremity bone tumors., Methods: The long-term follow-up study of the Childhood Cancer Survivor Study is a multiinstitutional cohort study comprising 14,054 individuals who have survived for 5 or more years after treatment for cancer diagnosed during childhood or adolescence. Baseline demographic and medical information were obtained. Six hundred ninety-four survivors had osteosarcoma or Ewing sarcoma of the lower extremity or pelvis and were classified by amputation status and by age at diagnosis. The median age at diagnosis was 14 years old with a median of 16 years of follow up since diagnosis. Demographic characteristics were used to analyze the rates of psychosocial outcomes., Results: Amputation status and age at diagnosis did not significantly influence any of the measured psychosocial outcomes. Education was a significant positive predictor of employment, having health insurance, and being currently in their first marriage. Male gender predicted ever being employed and female gender predicted having health insurance and marriage. When compared with siblings, amputees had significant deficits in education, employment, and health insurance., Conclusions: Overall, no differences between amputees and nonamputees were found. However, gender and education play a prominent role. When compared with siblings, amputees in this cohort may benefit from additional supports., (Copyright 2003 American Cancer Society.DOI 10.1002/cncr.11363)

Background: Neuroblastoma has many characteristics which suggest that preclinical detection might improve outcome. The Quebec Neuroblastoma Screening Project was initiated to determine whether mass screening could reduce mortality in a large cohort of infants. As an early endpoint, we report whether screening could reduce the incidence of poor-prognosis neuroblastoma in children with advanced-stage disease over 1 year of age., Methods: All 476,603 children born in the province of Quebec during the 5-year period of May 1, 1989, to April 30, 1994, were eligible for urinary assay of homovanillic acid and vanillylmandelic acid at 3 weeks and 6 months of age. Children with a positive screen were referred to one of four paediatric cancer centres in the province for uniform evaluation and treatment if necessary. Standardised incidence ratios (SIRs) were calculated for neuroblastoma in the province and two similar population-based controls, the state of Minnesota and the province of Ontario, during the same period of time and with similar ascertainment procedures., Findings: Compliance with screening in Quebec province was 91% at 3 weeks (n = 425,816) and 74% at 6 months (n = 349,706). Through July 31, 1995, with a follow-up of the birth cohort of 15-75 months, 118 cases of neuroblastoma were diagnosed, 43 detected preclinically by screening, 20 detected clinically before screening at 3 weeks of age, and 55 detected clinically after 3 weeks of age having normal screens (52) or never screened (3). Retrospective analysis of stored samples confirmed that 49 of 52 patients missed by screening had levels of catecholamine metabolites that were too low to be detected at 6 months or earlier. Based on US Surveillance, Epidemiology and End Results data, 54.5 cases of neuroblastoma would have been expected in Quebec province during the study period, for an SIR of 2.17 (95% CI 1.79-2.57, p < 0.0001). For the two control groups, 43 and 80 cases of neuroblastoma were detected, respectively, compared with 37.9 and 85.4 expected, overall SIR 1.00 (not significant). SIRs for Quebec province by age at diagnosis in yearly intervals show a marked increased incidence under 1 year of age (SIR 2.85, 2.26-3.50), with no reduction in incidence in subsequent years. Limiting analysis to only patients diagnosed over 1 year of age with advanced-stage disease, 22 cases were detected in Quebec province versus 14.4 expected (SIR 1.52, 0.95-2.23). Data in the two control groups show no significant increase or decrease in any-stage disease in children under or over the age of 1 year, except for an increase in early-stage disease in Minnesota children over 1 year: 10 versus 3.8 expected (SIR 2.67, 1.27-4.58)., Interpretation: Screening for neuroblastoma increases the incidence in infants without decreasing the incidence of unfavourable advanced-stage disease in older children. It is unlikely that screening for neuroblastoma in infants will reduce mortality for this disease.