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We propose a New Classification of Rare Cancer (NCRC) using the updated International Classification of Diseases for Oncology 3.2 coding system and World Health Organization Classification of Tumors 5th edition. We applied patient data recorded in the National Cancer Registry of Japan to the new classification, setting a cut\u2010off of a crude incidence rate of 6 cases\/100\u2009000\/year to define rare cancers, and developed a list of rare cancers in Japan from 2016 to 2019. The NCRC system identified various rare cancers, comprising 20.0% of all cancer diagnoses in this period. To examine this classification system's performance, we compared rare\/non\u2010rare labeling of cancers by the Surveillance of Rare Cancers in Europe (RARECARENet) project and NCRC system. Compared with cases using the RARECARENet classification in Europe, 69\u2009351 cases\/year (6.8%) switched status with our classification, with 45\u2009293 and 232\u2009109 cases (4 years) switching from rare and non\u2010rare, respectively. Major differences included diffuse large B\u2010cell lymphomas, some thyroid cancers, oral cavity and lip cancers, and squamous cell carcinoma of the uterine cervix. As the NCRC includes newly classified tumor entities, it warrants validation using other diverse cohorts.<\/jats:p>","DOI":"10.1111\/pin.70021","type":"journal-article","created":{"date-parts":[[2025,5,16]],"date-time":"2025-05-16T08:29:39Z","timestamp":1747384179000},"page":"291-309","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Proposal for a New Classification of Rare Cancers Adopting Updated Histological Tumor Types"],"prefix":"10.1111","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0232-8283","authenticated-orcid":false,"given":"Ryoko","family":"Rikitake","sequence":"first","affiliation":[{"name":"Division of Health Services Research Institute for Cancer Control, National Cancer Center Tokyo Japan"},{"name":"Rare Cancer Center, National Cancer Center Hospital Tokyo Japan"},{"name":"Department of Public Health and Health Policy Graduate School of Medicine, The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1788-559X","authenticated-orcid":false,"given":"Yasushi","family":"Yatabe","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology National Cancer Center Hospital Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoko","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Division of Health Services Research Institute for Cancer Control, National Cancer Center Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsunori","family":"Shimoi","sequence":"additional","affiliation":[{"name":"Rare Cancer Center, National Cancer Center Hospital Tokyo Japan"},{"name":"Department of Medical Oncology National Cancer Center Hospital Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shintaro","family":"Iwata","sequence":"additional","affiliation":[{"name":"Rare Cancer Center, National Cancer Center Hospital Tokyo Japan"},{"name":"Department of Musculoskeletal Oncology and Rehabilitation Medicine National Cancer Center Hospital Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasushi","family":"Goto","sequence":"additional","affiliation":[{"name":"Department of Thoracic Oncology National Cancer Center Hospital Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu","family":"Mizushima","sequence":"additional","affiliation":[{"name":"Division of Health Services Research Institute for Cancer Control, National Cancer Center Tokyo Japan"},{"name":"Department of Public Health and Health Policy Graduate School of Medicine, The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Kawai","sequence":"additional","affiliation":[{"name":"Rare Cancer Center, National Cancer Center Hospital Tokyo Japan"},{"name":"Department of Musculoskeletal Oncology and Rehabilitation Medicine National Cancer Center Hospital Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9933-2106","authenticated-orcid":false,"given":"Takahiro","family":"Higashi","sequence":"additional","affiliation":[{"name":"Division of Health Services Research Institute for Cancer Control, National Cancer Center Tokyo Japan"},{"name":"Department of Public Health and Health Policy Graduate School of Medicine, The University of Tokyo Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,5,16]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0092-8674(00)81683-9"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/003335491012500106"},{"key":"e_1_2_12_4_1","unstructured":"S.RecodeandS.Recodes[cited October 10 2024] https:\/\/seer.cancer.gov\/siterecode\/."},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2011.08.008"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(17)30445-X"},{"key":"e_1_2_12_7_1","unstructured":"Cancer list \u2013 RARECARENet. 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