{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T09:44:16Z","timestamp":1753868656599,"version":"3.41.2"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,1,26]],"date-time":"2024-01-26T00:00:00Z","timestamp":1706227200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Reports"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Mesenchymal neoplasms of the uterus encompass a diverse group of tumors, with varying characteristics and origins, collectively accounting for 8% of uterine malignancies. The most common variants include uterine leiomyosarcoma, low\u2010grade and high\u2010grade endometrial stromal sarcoma, adenosarcoma, and undifferentiated sarcoma. Clinical presentation is often nonspecific and can lead to delayed diagnosis. Uterine sarcomas are generally aggressive, resulting in poorer prognosis compared to carcinomas. Recent advances in molecular techniques, such as next\u2010generation sequencing (NGS), have led to the identification of new subtypes of uterine sarcomas, including <jats:italic>COL1A1::PDGFB<\/jats:italic> fusion\u2010associated fibrosarcoma, which has a specific chromosomal translocation t(17;22)(q22;q13). Imatinib, a tyrosine kinase inhibitor (TKI), is an effective treatment for dermatofibrosarcoma protuberans (DFSP), marked by this translocation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case<\/jats:title><jats:p>We present the case of a 42\u2010year\u2010old woman diagnosed with <jats:italic>COL1A1::PDGFB<\/jats:italic> fusion\u2010associated uterine fibrosarcoma. The patient underwent total hysterectomy and excision of the tumor, initially misdiagnosed as a low\u2010grade leiomyosarcoma. Subsequent histological examination, immunohistochemistry, and fluorescence in situ hybridization (FISH) confirmed the diagnosis. After 10 months, disease recurrence was detected, and Imatinib therapy was initiated at a dose of 400\u2009mg daily. An allergic reaction led to a temporary discontinuation, but upon resumption with appropriate medication, a positive radiological response was observed. The patient achieved a complete remission after 2\u2009years and is still on Imatinib treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p><jats:italic>COL1A1::PDGFB<\/jats:italic> fusion\u2010associated uterine fibrosarcoma is an extremely rare mesenchymal neoplasm. In a case we present herein, we treated a patient with imatinib as first\u2010line medical therapy. The patient is currently in complete remission after 37\u2009months from treatment start. To the best of our knowledge, this represents a unique observation. We also provide a detailed literature review of the published cases so far. Prospective case series are needed to further understand the natural history of these tumors and optimize treatment strategies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cnr2.1969","type":"journal-article","created":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T05:54:50Z","timestamp":1706334890000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["<i>COL1A1::PDGFB<\/i> fusion\u2010associated uterine fibrosarcoma: A case report and review of the literature"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-9172-7003","authenticated-orcid":false,"given":"Simone","family":"Rota","sequence":"first","affiliation":[{"name":"Department of Medical Oncology Fondazione IRCCS Istituto Nazionale dei Tumori di Milano  Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Franza","sequence":"additional","affiliation":[{"name":"Department of Medical 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