{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:02:25Z","timestamp":1792796545063,"version":"4.5.0"},"reference-count":28,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"9","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["JCO Precis Oncol"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:sec>\n            <jats:title>PURPOSE<\/jats:title>\n            <jats:p>\n              Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, representing &lt;0.5% of all ovarian tumors. We analyze the role of germline\n              <jats:italic>DICER1<\/jats:italic>\n              status in outcomes of ovarian SLCT.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>METHODS<\/jats:title>\n            <jats:p>\n              Patients with SLCT were enrolled in the International Pleuropulmonary Blastoma\/\n              <jats:italic>DICER1<\/jats:italic>\n              Registry and\/or the International Ovarian and Testicular Stromal Tumor Registry. Medical records were systematically abstracted, and those with known germline\n              <jats:italic>DICER1<\/jats:italic>\n              status were selected for analysis.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>RESULTS<\/jats:title>\n            <jats:p>\n              Of 162 patients with SLCT, 60% had a germline\n              <jats:italic>DICER1<\/jats:italic>\n              pathogenic or likely pathogenic (P\/LP) variant. The adjusted 3-year recurrence-free survival (RFS) was 87.2% (95% CI, 79.4 to 95.8) for patients with a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant compared with 78.1% (95% CI, 66.4 to 91.9) for those without a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant (\n              <jats:italic>P<\/jats:italic>\n              = .043). The adjusted 3-year and 5-year overall survival (OS) was 93.9% (95% CI, 87.3 to 100.0) for those with a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant compared with the 3-year OS of 91.3% (95% CI, 83.4 to 100.0) and the 5-year OS of 78.2% (95% CI, 63.8 to 95.9) for those without a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant (\n              <jats:italic>P<\/jats:italic>\n              = .021). Among patients with a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant, the risk of a subsequent, nonrecurrent event was 36.2% (95% CI, 21.4 to 48.1) within 10 years. Previous\/concurrent and subsequent neoplasms were rare among those without a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>CONCLUSION<\/jats:title>\n            <jats:p>\n              This cohort study of patients with SLCT demonstrated that those with germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variants had superior RFS and OS even when adjusting for other prognostic factors. Beyond prognostic implications of a germline\n              <jats:italic>DICER1<\/jats:italic>\n              P\/LP variant, germline testing helps identify patients at risk of subsequent neoplasms, including metachronous SLCT.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1200\/po-24-00902","type":"journal-article","created":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T19:59:13Z","timestamp":1745438353000},"update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":7,"title":["Prognostic Significance of Germline\n            <i>DICER1<\/i>\n            Pathogenic or Likely Pathogenic Variants in Outcomes of Ovarian Sertoli-Leydig Cell Tumor"],"prefix":"10.1200","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1611-4577","authenticated-orcid":true,"given":"Alexander T.","family":"Nelson","sequence":"first","affiliation":[{"name":"International Pleuropulmonary Blastoma\/DICER1 Registry, Children's Minnesota, Minneapolis, MN"},{"name":"International Ovarian and Testicular Stromal Tumor Registry, Children's Minnesota, Minneapolis, MN"},{"name":"Cancer and Blood Disorders, Children's Minnesota, Minneapolis, MN"},{"name":"Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2749-6136","authenticated-orcid":true,"given":"Dave","family":"Watson","sequence":"additional","affiliation":[{"name":"Research Institute, Children's Minnesota, Minneapolis, MN"},{"name":"Division of Clinical Trials and Biostatistics, Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2304-4631","authenticated-orcid":true,"given":"Kenneth S.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX"},{"name":"Children's Medical Center Research Institute, UT Southwestern Medical Center, Dallas, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9727-569X","authenticated-orcid":true,"given":"Damon R.","family":"Olson","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Children's Minnesota, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer N.","family":"Stall","sequence":"additional","affiliation":[{"name":"Hospital Pathology Associates, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8097-2437","authenticated-orcid":true,"given":"Kyle M.","family":"Devins","sequence":"additional","affiliation":[{"name":"James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Young","sequence":"additional","affiliation":[{"name":"James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2852-7387","authenticated-orcid":true,"given":"Junne","family":"Kamihara","sequence":"additional","affiliation":[{"name":"Pediatric Oncology, Dana-Farber\/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-6294-1733","authenticated-orcid":true,"given":"Paige H.R.","family":"Mallinger","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/DICER1 Registry, Children's Minnesota, Minneapolis, MN"},{"name":"International Ovarian and Testicular Stromal Tumor Registry, Children's Minnesota, Minneapolis, MN"},{"name":"Cancer and Blood Disorders, Children's Minnesota, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6274-2841","authenticated-orcid":true,"given":"Jung","family":"Kim","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2104-171X","authenticated-orcid":true,"given":"Jessica N.","family":"Hatton","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7990-1802","authenticated-orcid":true,"given":"Yoav H.","family":"Messinger","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/DICER1 Registry, Children's Minnesota, Minneapolis, MN"},{"name":"International Ovarian and Testicular Stromal Tumor Registry, Children's Minnesota, Minneapolis, MN"},{"name":"Cancer and Blood Disorders, Children's Minnesota, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4748-8552","authenticated-orcid":true,"given":"A. Lindsay","family":"Frazier","sequence":"additional","affiliation":[{"name":"Pediatric Oncology, Dana-Farber\/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8193-1488","authenticated-orcid":true,"given":"Douglas R.","family":"Stewart","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik T.","family":"Schneider","sequence":"additional","affiliation":[{"name":"Clinic of Pediatrics, Municipal Hospital Dortmund, University Witten\/Herdecke, Dortmund, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3371-3096","authenticated-orcid":true,"given":"Anne K.","family":"Harris","sequence":"additional","affiliation":[{"name":"International Pleuropulmonary Blastoma\/DICER1 Registry, Children's Minnesota, Minneapolis, MN"},{"name":"International Ovarian and Testicular Stromal Tumor Registry, Children's Minnesota, Minneapolis, MN"},{"name":"Cancer and Blood Disorders, Children's Minnesota, Minneapolis, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2007-5638","authenticated-orcid":true,"given":"Louis P.","family":"Dehner","sequence":"additional","affiliation":[{"name":"Department of Pathology and Immunology, Lauren V. 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