{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T09:10:47Z","timestamp":1788858647138,"version":"build-2803163510"},"reference-count":69,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>\n                      Li\u2010Fraumeni syndrome (LFS) is a cancer predisposition syndrome characterized by germline\n                      <jats:italic>TP53<\/jats:italic>\n                      mutations and increased risk of various malignancies. While rare uterine leiomyosarcomas (LMS) have been reported, other uterine smooth muscle tumours (uSMT) have not been studied in this context.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p>\n                      We describe clinicopathological features of uSMT from 10 LFS patients (four uSMT of uncertain malignant potential (STUMP), two leiomyomas with bizarre nuclei [LMBN] and four with only conventional leiomyomas [LM]) and next\u2010generation sequencing results of eight tumours. Among six p53\u2010aberrant tumours (four STUMPs, one LMBN, one LM) by immunohistochemistry (IHC), three STUMPs had biallelic\n                      <jats:italic>TP53<\/jats:italic>\n                      inactivation and three uSMT (two STUMPs, one LMBN) had loss of fumarate hydratase (FH) by IHC with inactivating\n                      <jats:italic>FH<\/jats:italic>\n                      variants. One patient with a p53\u2010aberrant, FH\u2010deficient LMBN had a concurrent p53\u2010wild\u2010type, FH\u2010proficient LM with a novel\n                      <jats:italic>ACTG2::BRAF<\/jats:italic>\n                      fusion. Two STUMPs with\n                      <jats:italic>TP53<\/jats:italic>\n                      biallelic inactivation and intact FH had additional alterations, one with LMS features, including chromosome instability,\n                      <jats:italic>ATRX,<\/jats:italic>\n                      and\n                      <jats:italic>RB1<\/jats:italic>\n                      alterations; the other with del(22q),\n                      <jats:italic>CYLD,<\/jats:italic>\n                      and\n                      <jats:italic>ELOC<\/jats:italic>\n                      mutations. The remaining two sequenced LM showed\n                      <jats:italic>MED12<\/jats:italic>\n                      alterations, one with del(22q). No recurrences were seen in nine patients with follow\u2010up and no LMS were diagnosed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      uSMTs exhibit a broad morphologic spectrum in LFS, and multiple molecular alterations may drive tumorigenesis, including\n                      <jats:italic>MED12<\/jats:italic>\n                      ,\n                      <jats:italic>FH<\/jats:italic>\n                      ,\n                      <jats:italic>TP53<\/jats:italic>\n                      ,\n                      <jats:italic>RB1<\/jats:italic>\n                      ,\n                      <jats:italic>ATRX<\/jats:italic>\n                      , and a novel\n                      <jats:italic>ACTG2::BRAF<\/jats:italic>\n                      fusion. Although some may be incidental, uSMT in this setting appears enriched for atypical morphology, FH deficiency, and aberrant p53 expression, suggesting an interplay between p53 and FH pathways in tumorigenesis.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/his.70201","type":"journal-article","created":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T13:33:01Z","timestamp":1781789581000},"page":"687-698","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinicopathological and molecular features of uterine smooth muscle tumours in patients with Li\u2010Fraumeni\u00a0syndrome"],"prefix":"10.1111","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9603-9783","authenticated-orcid":false,"given":"Lawrence H","family":"Lin","sequence":"first","affiliation":[{"name":"Department of Pathology, Brigham and Women's Hospital, Mass General Brigham Enterprise Harvard Medical 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USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3218-1075","authenticated-orcid":false,"given":"Zehra","family":"Ordulu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Brigham and Women's Hospital, Mass General Brigham Enterprise Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7631-6525","authenticated-orcid":false,"given":"David L","family":"Kolin","sequence":"additional","affiliation":[{"name":"Department of Pathology, Brigham and Women's Hospital, Mass General Brigham Enterprise Harvard Medical School  Boston Massachusetts 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