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Herein, 78 ACC were profiled using a combination of next generation sequencing (<jats:italic>n<\/jats:italic>\u2009=\u200976) and FISH (<jats:italic>n<\/jats:italic>\u2009=\u20099) to assess for <jats:italic>TERT<\/jats:italic> alterations. This data was combined with TCGA dataset (<jats:italic>n<\/jats:italic>\u2009=\u200991). A subset of borderline adrenocortical tumors (<jats:italic>n<\/jats:italic>\u2009=\u20095) and adrenocortical adenomas (<jats:italic>n<\/jats:italic>\u2009=\u20097) were also evaluated. The most common alteration involving the <jats:italic>TERT<\/jats:italic> gene involved gains\/amplifications, seen in 22.2% (37\/167) of cases. In contrast, \u201chotspot\u201d promoter mutations (C\u2009&gt;\u2009T promoter mutation at position -124, 7\/167 cases, 4.2%) and promoter rearrangements (2\/165, 1.2%) were rare. Recurrent co-alterations included 22q copy number losses seen in 24% (9\/38) of cases. Although no significant differences were identified in cases with and without <jats:italic>TERT<\/jats:italic> alterations pertaining to age at presentation, tumor size, weight, laterality, mitotic index and Ki67 labeling, cases with <jats:italic>TERT<\/jats:italic> alterations showed worse outcomes. Metastatic behavior was seen in 70% (28\/40) of cases with <jats:italic>TERT<\/jats:italic> alterations compared to 51.2% (65\/127, <jats:italic>p<\/jats:italic>\u2009=\u20090.04) of cases that lacked these alterations. Two (of 5) borderline tumors showed amplifications and no <jats:italic>TERT<\/jats:italic> alterations were identified in 7 adenomas. In the borderline group, 0 (of 4) patients with available follow up had adverse outcomes. We found that <jats:italic>TERT<\/jats:italic> alterations in ACC predominantly involve gene amplifications, with a smaller subset harboring \u201chotspot\u201d promoter mutations and rearrangements, and 70% of <jats:italic>TERT<\/jats:italic>-altered tumors are associated with metastases. Prospective studies are needed to validate the prognostic impact of these findings.<\/jats:p>","DOI":"10.1007\/s12022-021-09691-0","type":"journal-article","created":{"date-parts":[[2021,9,22]],"date-time":"2021-09-22T03:38:19Z","timestamp":1632281899000},"page":"304-314","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["TERT Copy Number Alterations, Promoter Mutations and Rearrangements in Adrenocortical Carcinomas"],"prefix":"10.1007","volume":"33","author":[{"given":"Sounak","family":"Gupta","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Won","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kalyani","family":"Chadalavada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gouri J.","family":"Nanjangud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying-Bei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hikmat A.","family":"Al-Ahmadie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samson W.","family":"Fine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sahussapont J.","family":"Sirintrapun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivian E.","family":"Strong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nitya","family":"Raj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane Reidy","family":"Lagunes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chad M.","family":"Vanderbilt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael F.","family":"Berger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Ladanyi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Snjezana","family":"Dogan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satish K.","family":"Tickoo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor E.","family":"Reuter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anuradha","family":"Gopalan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,22]]},"reference":[{"key":"9691_CR1","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1038\/nrm.2016.171","volume":"18","author":"J Maciejowski","year":"2017","unstructured":"Maciejowski J, de Lange T. 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