Epidemiological characteristics of many types of rare cancers are limited especially in Asia. Therefore, this study aimed to describe the burden and changing time trends of rare cancers in Hiroshima, ...Japan.
The internationally agreed RARECAREnet list of rare cancers was used to identify patients diagnosed with cancers from 2005 to 2015 who were registered in the Hiroshima Prefecture Cancer Registry. Quality indicators specific to rare cancers were assessed by cancer grouping. Crude incidence rates (IRs) and age-standardized rates (ASRs) were calculated for 216 single cancers (rare and common) included in the list. A joinpoint regression was used to analyze age distribution and time trends in the ASRs for 12 internationally agreed rare cancer families. Quality indicators, ASRs, and IRs in Japan were identified to examine IR differences and the effects on data accuracy.
The 231,328 cases were used to calculate the IRs of each cancer. Epithelial tumors in rare families increased with age, but nonepithelial tumors occurred at any age. The proportion of rare cancer families to total cancers was stable. The time trend for families of head and neck cancers (annual percent change and 95 % confidence interval: 2.4 %; 1.2–3.7 %), neuroendocrine tumors (6.6 %; 5.1–8.1 %), and hematological cancers (4.3 %; 3.2–5.5 %) markedly increased.
The ASRs of several rare cancers increased because of increased knowledge of these diseases, improved diagnostic techniques, and aggressive diagnoses.
•Rare cancer age distribution and changing time trends by cancer grouping were described using the RARECAREnet list in Hiroshima, Japan.•Burdens of epithelial cancers increased at middle age exponentially, but those of nonepithelial rare cancers by age were heterogeneous.•Incidence of rare cancers (e.g., head and neck, neuroendocrine, and hematological) increased by improving and aggressively pursuing diagnoses.
Rare cancers collectively account for a significant proportion of the overall cancer burden in Japan. We aimed to describe and examine the incidence of each rare cancer and the temporal changes using ...the internationally agreed rare cancer classification. Cancer cases registered in regional population-based cancer registries from 2011 to 2015 and the National Cancer Registry (NCR) from 2016 to 2018 were classified into 18 families, 68 Tier-1 cancer groupings, and 216 single cancer entities based on the RARECAREnet list. Crude incidence rates and age-standardized incidence rates (ASR) were calculated for Tier-1 and Tier-2 cancers. The annual percent change and the 95% and 99% confidence limits for annual ASR for each of the 68 Tier-1 cancers were estimated using the log-linear regression of the weighted least squares method. The differences in ASRs between 2011 and 2018 were evaluated as an absolute change. A total of 5,640,879 cases were classified into Tier-1 and Tier-2 cancers. The ASRs of 18 out of 52 Tier-1 cancers in the rare cancer families increased, whereas the ASR for epithelial tumors of gallbladder decreased. The ASRs of 6 out of the 16 Tier-1 cancers in the common cancer families increased, whereas those of epithelial tumors of stomach and liver decreased. There was no significant change in the incidence of the other 40 Tier-1 cancers. The incidence of several cancers increased due to the dissemination of diagnostic concepts, improved diagnostic techniques, changes in coding practice, and the initiation of the NCR.
Objective To investigate the degree to which ionising radiation confers risk of mortality from heart disease and stroke.Design Prospective cohort study with more than 50 years of follow-up.Setting ...Atomic bomb survivors in Hiroshima and Nagasaki, Japan.Participants 86 611 Life Span Study cohort members with individually estimated radiation doses from 0 to >3 Gy (86% received <0.2 Gy).Main outcome measures Mortality from stroke or heart disease as the underlying cause of death and dose-response relations with atomic bomb radiation.Results About 9600 participants died of stroke and 8400 died of heart disease between 1950 and 2003. For stroke, the estimated excess relative risk per gray was 9% (95% confidence interval 1% to 17%, P=0.02) on the basis of a linear dose-response model, but an indication of possible upward curvature suggested relatively little risk at low doses. For heart disease, the estimated excess relative risk per gray was 14% (6% to 23%, P<0.001); a linear model provided the best fit, suggesting excess risk even at lower doses. However, the dose-response effect over the restricted dose range of 0 to 0.5 Gy was not significant. Prospective data on smoking, alcohol intake, education, occupation, obesity, and diabetes had almost no impact on the radiation risk estimates for either stroke or heart disease, and misdiagnosis of cancers as circulatory diseases could not account for the associations seen.Conclusion Doses above 0.5 Gy are associated with an elevated risk of both stroke and heart disease, but the degree of risk at lower doses is unclear. Stroke and heart disease together account for about one third as many radiation associated excess deaths as do cancers among atomic bomb survivors.
PDF