{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T15:23:24Z","timestamp":1792423404532,"version":"4.5.0"},"reference-count":17,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2023,6,1]],"date-time":"2023-06-01T00:00:00Z","timestamp":1685577600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["P30CA015704"],"award-info":[{"award-number":["P30CA015704"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Malignant hemangioendothelioma is an endothelial cancer with heterogeneous clinical behavior that can range from indolent to aggressive, of which the majority are epithelioid (EHE). Its incidence and demographics have not been previously well defined in a large cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective analysis used the US Cancer Statistics National Program of Cancer Registries \u2013 Surveillance Epidemiology End Results (SEER) combined database to identify patients in the US newly diagnosed with hemangioendothelioma between the years of 2001 and 2017 (<jats:italic>n<\/jats:italic>\u2009=\u20091986). Survival analyses were performed on a subset of patients within the SEER\u201018 database with survival information available (<jats:italic>n<\/jats:italic>\u2009=\u2009417). Outcomes included incidence, demographics of patients newly diagnosed with hemangioendothelioma, extent of disease at presentation, and overall survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The incidence of hemangioendothelioma in the US is 0.4 cases per million person\u2010years. Although cases rose to 122 newly diagnosed in the year 2017 (90 EHE, 32 other hemangioendothelioma), incidence rates were stable. Skin and connective tissues were the most common presenting sites (33.4%), followed by liver (24.5%), lung (17.6%), and bone (12.5%). Median age at diagnosis was 55\u2009years; 27.2% of patients were pediatric, adolescent, or young adult (&lt;40\u2009years). At presentation, 36.4% of patients had localized disease; 21.6% presented with regional and 41.7% with distant metastases. Observed survival at 3\u2009years was 79.7%, 70.7%, and 46.0% for patients presenting with local, regional, and distant disease and most deaths occurred within the first 2\u2009years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Malignant hemangioendothelioma is ultra\u2010rare but meaningfully impacts affected patients. These data may provide benchmarks for comparison of new approaches to hemangioendothelioma therapy and highlight poor survival outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cam4.6181","type":"journal-article","created":{"date-parts":[[2023,6,1]],"date-time":"2023-06-01T12:20:54Z","timestamp":1685622054000},"page":"15101-15106","source":"Crossref","is-referenced-by-count":19,"title":["Incidence, demographics, and survival of malignant hemangioendothelioma in the United States"],"prefix":"10.1002","volume":"12","author":[{"given":"Kelly G.","family":"Paulson","sequence":"first","affiliation":[{"name":"Medical Oncology Providence\u2010Swedish Cancer Institute  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6080-9336","authenticated-orcid":false,"given":"Vinod","family":"Ravi","sequence":"additional","affiliation":[{"name":"Sarcoma Medical Oncology MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian P.","family":"Rubin","sequence":"additional","affiliation":[{"name":"Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Park","sequence":"additional","affiliation":[{"name":"Medical Oncology Providence\u2010Swedish Cancer Institute  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth T.","family":"Loggers","sequence":"additional","affiliation":[{"name":"Clinical Research Division Fred Hutchinson Cancer Center  Seattle Washington USA"},{"name":"Division of Medical Oncology University of Washington  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7789-5442","authenticated-orcid":false,"given":"Lee D.","family":"Cranmer","sequence":"additional","affiliation":[{"name":"Clinical Research Division Fred Hutchinson Cancer Center  Seattle Washington USA"},{"name":"Division of Medical Oncology University of Washington  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0753-9282","authenticated-orcid":false,"given":"Michael J.","family":"Wagner","sequence":"additional","affiliation":[{"name":"Clinical Research Division Fred Hutchinson Cancer Center  Seattle Washington USA"},{"name":"Division of Medical Oncology University of Washington  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,6]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0246958"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1155\/2022\/2349991"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3002409"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20886"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-019-0368-8"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-021-00879-7"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22073"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.esmoop.2021.100170"},{"key":"e_1_2_9_10_1","unstructured":"Centers for Disease Control and Prevention and National Cancer InstituteNational Program of Cancer Registries and Surveillance Epidemiology and End Results SEER*Stat Database: NPCR and SEER Incidence \u2013 U.S. Cancer Statistics 2001\u20132017 Public Use Research Database 2019 submission (2001\u20132017) United States Department of Health and Human Services Centers for Disease Control and Prevention and National Cancer Institute. 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