{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T10:58:27Z","timestamp":1790333907110,"version":"4.1.0"},"reference-count":14,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T00:00:00Z","timestamp":1790294400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>\n                    <jats:italic>DICER1<\/jats:italic>\n                    syndrome is a hereditary tumor predisposition syndrome caused by pathogenic germline variants in\n                    <jats:italic>DICER1<\/jats:italic>\n                    and is characterized by multi-organ susceptibility; sarcomas remain uncommon within this spectrum. We report a 2-year-old girl with anaplastic sarcoma of the kidney (ASK). Molecular testing identified a germline\n                    <jats:italic>DICER1<\/jats:italic>\n                    c.3300dupA frameshift variant (p.S1101Ifs*3), inherited from her father, and a tumor-associated c.5125G&amp;gt;A missense variant (p.D1709N). The child had neonatal hypothyroidism and language developmental delay. She underwent complete tumor resection followed by 27 weeks of chemotherapy using a Stage I clear cell sarcoma of the kidney (CCSK)-based regimen. At the most recent follow-up, she was 3 years off therapy with no evidence of tumor recurrence or distant metastasis. This case adds to the limited clinicopathologic and molecular descriptions of\n                    <jats:italic>DICER1<\/jats:italic>\n                    -associated ASK and supports integrated histopathologic and molecular evaluation, familial\n                    <jats:italic>DICER1<\/jats:italic>\n                    assessment, genetic counseling, and long-term syndrome-directed surveillance.\n                  <\/jats:p>","DOI":"10.3389\/fonc.2026.1863816","type":"journal-article","created":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T09:58:45Z","timestamp":1790330325000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: DICER1 mutation-associated anaplastic sarcoma of the kidney in a child"],"prefix":"10.3389","volume":"16","author":[{"given":"Meng","family":"Li","sequence":"first","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital (WCSUH)-Tianfu, Sichuan Provincial Children's Hospital","place":["Meishan, Sichuan, China"]},{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoqian","family":"He","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xia","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ju","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cheng-qi","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital (WCSUH)-Tianfu, Sichuan Provincial Children's Hospital","place":["Meishan, Sichuan, China"]},{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, Sichuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,9,25]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1038\/35053110","article-title":"Role for a bidentate ribonuclease in the initiation step of RNA interference","volume":"409","author":"Bernstein","year":"2001","journal-title":"Nature"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0504834102","article-title":"The RNaseIII enzyme Dicer is required for morphogenesis but not patterning of the vertebrate 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