{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T20:39:36Z","timestamp":1789159176640,"version":"build-2803163510"},"reference-count":36,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T00:00:00Z","timestamp":1765584000000},"content-version":"vor","delay-in-days":12,"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"}]},{"DOI":"10.13039\/100019543","name":"Kristen Ann Carr Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100019543","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":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    The incidence and impact of telomerase reverse transcriptase (TERT) gene fusions in sarcomas remain poorly defined. To date only two cases have been reported, both displaying spindle cell liposarcoma morphology. In this study, we identified four soft tissue spindle cell neoplasms harboring in\u2010frame TERT fusions using targeted RNA sequencing and provide an in\u2010depth characterization of their histologic spectrum, immunoprofile and genomic landscape. Clinically, there was one female and three males, with a wide age range at diagnosis (47\u201379\u2009years, mean 60\u2009years). The tumors showed a diverse anatomic location, including lower extremity, trunk and retroperitoneum. Histologically, they exhibited a predominantly monomorphic spindle cell phenotype, in keeping with fibroblastic\/myofibroblastic differentiation. Three tumors were low\u2010grade, while one displayed increased cellularity and mitotic activity in keeping with a high\u2010grade lesion. Two tumors showed CD34 positivity, while the other two were characterized by actin positivity, with one showing nuclear \u03b2\u2010catenin expression. All cases harbored in\u2010frame\n                    <jats:italic>TERT<\/jats:italic>\n                    fusions, involving three different partners, such as\n                    <jats:italic>TRIO<\/jats:italic>\n                    in two cases, and\n                    <jats:italic>AHRR<\/jats:italic>\n                    and\n                    <jats:italic>SUGCT<\/jats:italic>\n                    in one case each. Two of the three cases tested by targeted NGS panels showed in addition\n                    <jats:italic>TERT<\/jats:italic>\n                    gene amplification and\n                    <jats:italic>CDKN2A<\/jats:italic>\n                    deletion, including the high\u2010grade lesion. To define more broadly the recurrent potential and histotype distribution of\n                    <jats:italic>TERT<\/jats:italic>\n                    fusions we also investigated our sarcoma genomic database for this genetic alteration. Results showed that a small subset of well\u2010defined sarcoma types harbored\n                    <jats:italic>TERT<\/jats:italic>\n                    fusions, typically coexisting with numerous other genomic alterations. Thus, our findings suggest that in\u2010frame\n                    <jats:italic>TERT<\/jats:italic>\n                    fusions may drive the pathogenesis of a group of mostly low\u2010grade unclassified sarcomas with fibroblastic\/myofibroblastic differentiation. In contrast,\n                    <jats:italic>TERT<\/jats:italic>\n                    fusions may also be detected in other common sarcoma types co\u2010occurring with numerous genomic alterations, likely representing a secondary driver event.\n                  <\/jats:p>","DOI":"10.1002\/gcc.70097","type":"journal-article","created":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T09:02:57Z","timestamp":1765616577000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Expanding the Morphologic and Molecular Spectrum of Spindle Cell Tumors Associated With\n                    <scp>TERT<\/scp>\n                    Fusions"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1854-1481","authenticated-orcid":false,"given":"Susan Swee\u2010Shan","family":"Hue","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"},{"name":"Department of Pathology National University Hospital  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla","family":"Saoud","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorukhan","family":"Bahceci","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liliana","family":"Villafania","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamal","family":"Benhamida","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine 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 and Laboratory Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201610111"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.589929"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2011.02.013"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1229259"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1303607110"},{"issue":"22","key":"e_1_2_9_7_1","first-page":"6230","article-title":"Frequent Amplification of the Telomerase Reverse Transcriptase Gene in Human Tumors","volume":"60","author":"Zhang A.","year":"2000","journal-title":"Cancer 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