{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T15:53:42Z","timestamp":1792079622075,"version":"4.4.0"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,6,10]],"date-time":"2025-06-10T00:00:00Z","timestamp":1749513600000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P50 CA217694"],"award-info":[{"award-number":["P50 CA217694"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30 CA008748"],"award-info":[{"award-number":["P30 CA008748"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017050","name":"Cycle for Survival","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>Bone angiosarcoma (B\u2010AS) is an exceedingly rare and aggressive vascular neoplasm, with limited therapeutic options and poor outcomes. Unlike the more prevalent clinical subsets (breast, head and neck), the pathogenesis of B\u2010AS remains poorly defined, with no targeted therapeutic strategies available. Moreover, the often delays in diagnosis, either radiographically or pathologically, as well as the common multifocal presentation, further impact the feasibility of surgical management, contributing to lower survival rates. In this study, we investigated the clinicopathologic and molecular characteristics of B\u2010AS to better define prognostic factors influencing outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>This retrospective study analyzed 22 cases of B\u2010AS managed at a single tertiary cancer center from 1998 to 2024. Clinical and pathologic data were extracted through chart reviews and re\u2010assessment of radiology and histology. Molecular characterization was performed in a subset using targeted next\u2010generation sequencing (NGS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The cohort included 14 males and eight females (median age: 64.5\u2009years), with tumors mostly involving femur, pelvis, and spine. Twelve (55%) patients presented with disease limited to the bone, either solitary or multifocal, while 10 (45%) patients presented in addition with extraskeletal metastases at diagnosis. The skeletal distribution included six (27%) solitary bone lesions, with the remaining 16 being multifocal (four contiguous, twelve disseminated). A surgical procedure for the bone lesions was performed in 73% of cases, varying from intralesional curetting to limb amputation. Half of the patients received radiation, and 73% chemotherapy. By molecular profiling, all tumors showed a low tumor mutational burden (TMB), with the most frequent alterations being <jats:italic>KDR<\/jats:italic> mutations and <jats:italic>MYC<\/jats:italic> amplifications. Age and chemotherapy were significantly associated with improved overall survival (OS) (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.005); however, the 3\u2010year OS was only 30%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite the multidisciplinary approach and orthopedic oncology expertise from a tertiary cancer center, the prognosis for B\u2010AS remains poor. Although limited in number, the molecular profiling revealed overlapping genomic alterations with other clinical subsets of AS, having the potential for individualized patient management.<\/jats:p><\/jats:sec>","DOI":"10.1002\/gcc.70056","type":"journal-article","created":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T07:19:53Z","timestamp":1751008793000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Clinicopathologic and Molecular Analysis of Primary Angiosarcoma of Bone\u2014A Single Institution Experience"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1588-9757","authenticated-orcid":false,"given":"Takeshi","family":"Hirose","sequence":"first","affiliation":[{"name":"Department of Pathology Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hsin\u2010Yi","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Pathology Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert A.","family":"Lefkowitz","sequence":"additional","affiliation":[{"name":"Department of Radiology Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0802-1186","authenticated-orcid":false,"given":"John","family":"Healey","sequence":"additional","affiliation":[{"name":"Department of Surgery, Orthopedic Service Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9717-8205","authenticated-orcid":false,"given":"Cristina R.","family":"Antonescu","sequence":"additional","affiliation":[{"name":"Department of Pathology Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,6,10]]},"reference":[{"key":"e_1_2_10_2_1","volume-title":"Soft Tissue and Bone Tumours","author":"WHO Classification of Tumours Editorial Board","year":"2020"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-020-66579-5"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/COC.0b013e31827defa1"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000582"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22219"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41591-019-0749-z"},{"key":"e_1_2_10_8_1","volume-title":"Angiosarcoma of Bone: Solitary and Multifocal Variants","author":"Mirra J. M.","year":"1989"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1006\/bcmd.1996.0101"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1080\/13577149778290"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmoldx.2014.12.006"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm.3729"},{"key":"e_1_2_10_13_1","volume-title":"Ggplot2 Elegant Graphics for Data Analysis","author":"Wickham H.","year":"2016"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btw313"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002560000261"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199306000-00009"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2018-0395-RS"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejrad.2010.06.052"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiographics.15.4.7569134"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13244-021-01129-9"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(03)00249-1"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22898"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20886"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22073"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-019-0368-8"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2921"}],"container-title":["Genes, Chromosomes and Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/gcc.70056","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T09:58:44Z","timestamp":1754906324000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/gcc.70056"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6]]},"references-count":25,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,6]]}},"alternative-id":["10.1002\/gcc.70056"],"URL":"https:\/\/doi.org\/10.1002\/gcc.70056","archive":["Portico"],"relation":{},"ISSN":["1045-2257","1098-2264"],"issn-type":[{"value":"1045-2257","type":"print"},{"value":"1098-2264","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6]]},"assertion":[{"value":"2025-05-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-05-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70056"}}