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This study quantified incidence for each rare cancer and assessed temporal changes using the internationally agreed rare cancer classification. Cases were drawn from regional population-based cancer registries for 2011–2015 and the National Cancer Registry for 2016–2018, mapped to 18 families, 68 tier-1 groups, and 216 single entities from the RARECAREnet list. Crude and age-standardized incidence rates were estimated, with annual percent change and confidence limits computed via log-linear regression, and absolute differences assessed between 2011 and 2018. Results showed increases in 18/52 tier-1 cancers in rare families, decreases in specific epithelial tumors, and no significant change for remaining cancers. Trends were attributed to changes in diagnostic concepts, techniques, coding practice, and NCR initiation.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":40,"@type":76,"position":81},"https://docshare.wps.com/document/research-report/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/time-trend-analysis-of-rare-cancer-incidence-20112018-nationwide-population-based-cancer-registries-in-japan/330957/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/time-trend-analysis-of-rare-cancer-incidence-20112018-nationwide-population-based-cancer-registries-in-japan/330957.png","ImageObject",300,407,{"name":92,"@type":93},"Aria","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-23","2026-09-21",true,{"@type":102,"interactionType":103,"userInteractionCount":39},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What data sources were used to study rare cancer incidence from 2011 to 2018?","Question",{"text":112,"@type":113},"Cancer cases were registered in regional population-based cancer registries from 2011–2015, and in the National Cancer Registry from 2016–2018 in Japan.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How were cancers classified for incidence and trend analyses?",{"text":117,"@type":113},"Cases were classified using the internationally agreed rare cancer classification: 18 families, 68 Tier-1 groupings, and 216 single cancer entities based on the RARECAREnet list.",{"name":119,"@type":110,"acceptedAnswer":120},"Which metrics quantified changes in incidence over time?",{"text":121,"@type":113},"Crude incidence rates and age-standardized incidence rates (ASR) were calculated, and annual percent change with 95% and 99% confidence limits was estimated using log-linear regression of weighted least squares.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},330957,1790034347,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":39,"category_name":40,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":39,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},2336464648322,"https://ap-avatar.wpscdn.com/avatar/2200025388227c56fec?_k=1778556882303663488","DOI: 10. 1111/cas.16176  \nOR I G I NAL ART I C L E  \nTime trend analysis of rare cancer incidence 2011–2018: Nationwide population-based cancer registries in Japan  \nHiromi Sugiyama1  | Manami Konda1 | Kumiko Saika2  | Tomohiro Matsuda2   \n1Department of Epidemiology, Radiation Effects Research Foundation, Hiroshima, Japan  \n2Division of International Health Policy Research, Institution for Cancer Control, National Cancer Center, Tokyo, Japan  \nCorrespondence  \nHiromi Sugiyama, Department of Epidemiology, Radiation Effects Research Foundation, 5-2 Hijiyama Park, Minamiku, Hiroshima City, Hiroshima, 732-0815, Japan.  \nEmail: [sugi@rerf.or.jp](sugi@rerf.or.jp)  \nFunding information  \nMinistry of Health, Labour and Welfare  \nAbstract  \nRare 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.  \nK E Y WO R D S  \nincidence, National Cancer Registry, population-based cancer registry, rare cancer, time trend  \n1 | INTRODUCTION  \nSince 2012, there has been an impetus on the control of rare cancersin Japan based on the Basic Plan to Cancer Control Program.1 The cancer incidence statistics based on the PBCRs have been provided  \naccording to ICD-10 to ensure comparability with cancer mortality.2 However, the ICD-10 classification does not include definitions for each rare cancer with a combination of topography and histological type. Thus, there was a paucity of comprehensive epidemiological analyses focusing on rare cancers until around 2010.  \nAbbreviations: APC, annual percent change; ASR, age-standardized incidence rate; CI, confidence interval; CL, confidence limit; CNS, central nervous system; CR, crude rate; DCO, death certificate only; EU, European Union; FL, follicular lymphoma; GIST, gastrointestinal stromal tumor; HBV, hepatitis B virus; HCV, hepatitis C virus; ICD-10, International Classification of Diseases, 10th Revision; ICD-O-3, International Classification of Disease for Oncology, 3rd Edition; MDS, myelodysplastic syndrome; MI, mortality incidence; NCR, National Cancer Registry; NET, neuroendocrine tumor; NOS, not otherwise specified; PBCR, population-based cancer registry; PNET, pancreatic neuroendocrine tumor; SEER, Surveillance Epidemiology and End Results.  \nThis is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.  \n© 2024 The Authors. Cancer Science publi","cbCaipGrJGUAtdtK","https://ap.wps.com/l/cbCaipGrJGUAtdtK","pdf",1517785,27,"English","# Abstract\n# Introduction\n# Methods\n## Population-based regional cancer registries and the NCR in Japan\n## Data classification and incidence rate estimation","[{\"question\":\"What data sources were used to study rare cancer incidence from 2011 to 2018?\",\"answer\":\"Cancer cases were registered in regional population-based cancer registries from 2011–2015, and in the National Cancer Registry from 2016–2018 in Japan.\"},{\"question\":\"How were cancers classified for incidence and trend analyses?\",\"answer\":\"Cases were classified using the internationally agreed rare cancer classification: 18 families, 68 Tier-1 groupings, and 216 single cancer entities based on the RARECAREnet list.\"},{\"question\":\"Which metrics quantified changes in incidence over time?\",\"answer\":\"Crude incidence rates and age-standardized incidence rates (ASR) were calculated, and annual percent change with 95% and 99% confidence limits was estimated using log-linear regression of weighted least squares.\"}]","Time trend analysis of rare cancer incidence 2011–2018: Nationwide population-based cancer registries in Japan | PDF",1789990871,68]