Abstract
Background
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.
Methods
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.
Results
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.
Conclusion
The ASRs of several rare cancers increased because of increased knowledge of these diseases, improved diagnostic techniques, and aggressive diagnoses.
Highlights
- • 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.
Introduction
Rare cancers imply cancers with which the number of patients is small in a population. However, the proportion of the rare cancers to total is not small. The RARECAREnet defined the threshold for rare cancer as an incidence rate (IR) of less than 6/100,000 per year on the basis of the population level in Europe . Rare cancers were observed 24 % of the total cancer incidence rate (IR) in Europe ; 19–25 %, depending on race, in the USA ; 20.4 % in Brazil ; and 15 % in Japan . Using the updated RARECAREnet list , a collaborative study conducted by RARECAREnet Asia measured and compared the rare cancer burdens in Japan (16.3 %), Korea (23.7 %), Taiwan (24.0 %), and the EU (22.2 %) . Furthermore, the study showed that cancers that are rare in Europe are also rare in Asia .
In Japan, the third-term Basic Plan to Promote Cancer Control Program approved in 2018 has aimed for all the people, including patients with cancer, to learn about and overcome cancer, including rare cancers. However, epidemiological knowledge of the characteristics of each rare cancer is limited. The age distribution and time trends of rare cancers in Asian countries have not yet been provided. A sufficient number of pathologically confirmed and coded cases with detailed morphology is essential to identify and study their detailed characteristics . The high-quality data of the Hiroshima Prefecture Cancer Registry (HPCR), enhanced by the Hiroshima Tumor Tissue Registry (HTTR), a population-based pathological tissue registry, enable the description of rare cancer characteristics. This study aimed to measure the IR of rare cancers according to subtype, as defined by the RARECAREnet list, to determine the age distribution and incidence time trends in the Hiroshima Prefecture.
Hiroshima City, located in the west of Hiroshima Prefecture, experienced atomic bombing in 1945. However, the atomic bomb radiation was high-dose rate radiation, and the radiation-affected geographic area was limited. The proportion of survivors exposed to atomic bomb radiation greater than 5 mGy was estimated to be 0.45 % among the atomic bomb survivors’ cohort of the Life Span Study (LSS) compared to the total population of Hiroshima Prefecture in 2009 . Therefore, the cancer incidence related to atomic bomb radiation in Hiroshima Prefecture is low.
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