{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T17:02:20Z","timestamp":1778691740360,"version":"3.51.4"},"reference-count":54,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T00:00:00Z","timestamp":1761091200000},"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>The low-grade endometrial stromal sarcoma (LG-ESS) represents a slowly proliferating subtype of endometrial stromal tumor but exhibits a preference for late-stage recurrence. Incidence of invasion into major blood vessels and the heart is exceedingly uncommon in this particular neoplasm. We describe a patient with recurrent LG-ESS whose tumor mainly involved the right ovary and right kidney and extended from the ovarian vein to the right atrium. Furthermore, we comprehensively review existing literature on 35 patients exhibiting similar manifestations. In these 36 cases, tumor thrombus primarily extended to the inferior vena cava (n=17), with a minority extending to the right atrium (n=12), while no cases were detected when the tumor thrombus had just extended to the iliac blood vessels, indicating that LG-ESS exhibits an insidious onset during early-stage vascular invasion. It is advisable for comprehensive examination and diligent follow-up in high-risk patients. 19 cases with complete tumor resection were followed up, and all except two patients remained alive with no evidence of disease. Among the 5 cases of mortality, 2 cases did not undergo surgical intervention, while in 3 cases where surgery was performed, complete tumor resection could not be achieved. The causes of death in these patients were related to disease progression or concurrent cardiovascular events. These findings underscore the importance of cytoreductive surgery adhering to the tumor-free principle, or at least removing the lesions in the major blood vessels and heart to prevent acute embolism and sudden demise, which usually requires multidisciplinary teamwork.<\/jats:p>","DOI":"10.3389\/fonc.2025.1671080","type":"journal-article","created":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T04:18:08Z","timestamp":1761106688000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Multidisciplinary management of intracardiac tumor thrombus in low-grade endometrial stromal sarcoma: case report and literature review"],"prefix":"10.3389","volume":"15","author":[{"given":"Hanke","family":"Zhang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jinhua","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chao","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Si","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miao","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoyan","family":"Xin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongbo","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaowu","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.asjsur.2022.12.012","article-title":"High-grade endometrial stromal sarcoma with an extremely rapid progression: A rare case report","volume":"46","author":"Dong","year":"2023","journal-title":"Asian J Surg"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.3390\/cancers17111893","article-title":"Endometrial stromal sarcoma: an update","volume":"17","author":"Ricotta","year":"2025","journal-title":"Cancers (Basel)"},{"key":"B3","first-page":"4","article-title":"Female genital tumours: who classification of tumours","volume-title":"WHO Classification of Tumours","author":"Moch","year":"2020"},{"key":"B4","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1186\/s12957-021-02474-1","article-title":"Primary low-grade extrauterine endometrial stromal sarcoma: analysis of 10 cases with a review of the literature","volume":"20","author":"Wu","year":"2022","journal-title":"World J Surg Oncol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1097\/pas.0000000000002428","article-title":"Low-grade endometrial stromal sarcoma: clinicopathologic and prognostic features in a cohort of 102 tumors","volume":"49","author":"Devins","year":"2025","journal-title":"Am J Surg Pathol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2022.01.004","article-title":"Endometrial stromal tumors: diagnostic updates and challenges","volume":"39","author":"Niu","year":"2022","journal-title":"Semin Diagn Pathol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.101132598","article-title":"Frequent fusion of the Jazf1 and Jjaz1 genes in endometrial stromal tumors","volume":"98","author":"Koontz","year":"2001","journal-title":"Proc Natl Acad Sci"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2022.110889","article-title":"Jazf1-Suz12 dysregulates Prc2 function and gene expression during cell differentiation","volume":"39","author":"Tavares","year":"2022","journal-title":"Cell Rep"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22845","article-title":"A novel Mbtd1-Phf1 gene fusion in endometrial stromal sarcoma: A case report and literature review","volume":"59","author":"Han","year":"2020","journal-title":"Genes Chromosomes Cancer"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.24969","article-title":"Identification of an Epc2-Phf1 fusion transcript in low-grade endometrial stromal sarcoma","volume":"9","author":"Brunetti","year":"2018","journal-title":"Oncotarget"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2014.01.030","article-title":"Meaf6\/Phf1 is a recurrent gene fusion in endometrial stromal sarcoma","volume":"347","author":"Micci","year":"2014","journal-title":"Cancer Lett"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.5384","article-title":"Genomic landscape of endometrial stromal sarcoma of uterus","volume":"6","author":"Choi","year":"2015","journal-title":"Oncotarget"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(20)31467-7","article-title":"Intracardiac extension of Malignant uterine tumors. 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