{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T09:52:51Z","timestamp":1753869171329,"version":"3.41.2"},"reference-count":34,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T00:00:00Z","timestamp":1725840000000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"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":[[2024,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Angiosarcomas of the kidney and adrenal gland are rare, highly aggressive vascular neoplasms. Their genomic profile has not been systematically studied to date. We report the clinicopathologic and molecular features of six angiosarcomas centered in the kidney\/adrenal gland. All patients were male adults, ranging from 58 to 77\u2009years of age. Tumor sizes ranged from 2.5 to 22.5\u2009cm. Half of the cases demonstrated hot spot mutations in the <jats:italic>KDR<\/jats:italic> gene, while one\u2010third demonstrated mutations in the <jats:italic>PIK3CA<\/jats:italic> gene; both of these gene alterations being previously described, preferentially in breast angiosarcomas. In addition, two cases each demonstrated <jats:italic>BRIP1<\/jats:italic> gene amplification, <jats:italic>CTNNB1<\/jats:italic> and <jats:italic>ETV6<\/jats:italic> mutations, which have not been previously reported in angiosarcoma. Notably, molecular studies were critical in establishing the correct diagnoses in three cases: one was an epithelioid angiosarcoma originally misdiagnosed as metastatic adenocarcinoma to the adrenal gland, the second was a vasoformative angiosarcoma that mimicked hemangioma, and the third was a collision tumor between a high\u2010grade angiosarcoma and a chromophobe renal cell carcinoma which was originally diagnosed as a sarcomatoid renal cell carcinoma. In summary, angiosarcomas of the kidney and adrenal gland have a high frequency of recurrent genetic alterations, some of them being shared with other angiosarcoma subtypes, while other appear to be novel. In particular, activating hot spot <jats:italic>KDR<\/jats:italic> and <jats:italic>PIK3CA<\/jats:italic> mutations represent potential therapeutic targets for these highly aggressive cancers.<\/jats:p>","DOI":"10.1002\/gcc.23268","type":"journal-article","created":{"date-parts":[[2024,9,9]],"date-time":"2024-09-09T13:07:26Z","timestamp":1725887246000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Molecular Analysis of Renal\/Adrenal Angiosarcomas Reveals High Frequency of Recurrent Genetic Alterations"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8797-9474","authenticated-orcid":false,"given":"Pedram","family":"Argani","sequence":"first","affiliation":[{"name":"Department of Pathology The Johns Hopkins Medical Institutions  Baltimore Maryland USA"},{"name":"Department of Oncology The Johns Hopkins Medical Institutions  Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla","family":"Saoud","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan\u2010Kettering Cancer Center  New York New\u00a0York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9717-8205","authenticated-orcid":false,"given":"Cristina\u00a0R.","family":"Antonescu","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan\u2010Kettering Cancer Center  New York New\u00a0York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,9,9]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.14458"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2018.10.002"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2013.176"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.6137"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2921"},{"key":"e_1_2_9_7_1","first-page":"1","article-title":"Targeted Massively Parallel Sequencing of Angiosarcomas Reveals Frequent Activation of the Mitogen Activated Protein Kinase Pathway","volume":"3","author":"Murali R.","year":"2015","journal-title":"Oncotarget"},{"key":"e_1_2_9_8_1","first-page":"1","article-title":"Immunological and Genomic Analysis Reveals Clinically Relevant Distinctions Between Angiosarcoma Subgroups","volume":"30","author":"Van Ravensteijn S. 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