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In the latest WHO Classification of Tumors of the Female Reproductive System (2020), this entity has been assigned to a separate category based on its distinct morphological and molecular-genetic features. Epithelioid inflammatory myofibroblastic sarcoma represents an exceptionally rare variant of inflammatory myofibroblastic tumor in the female genital tract. ALK gene rearrangements are characteristic for inflammatory myofibroblastic tumors and provide opportunities for targeted therapy with ALK inhibitors. This article presents a rare clinical case of uterine inflammatory myofibroblastic sarcoma. The case highlights the importance of accurate morphological and molecular verification of the diagnosis and demonstrates the effectiveness of second-generation ALK inhibitors in managing aggressive forms of inflammatory myofibroblastic tumors of uterine localization.<\/jats:p>","DOI":"10.17116\/patol20268805166","type":"journal-article","created":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T08:16:23Z","timestamp":1792138583000},"page":"66","source":"Crossref","is-referenced-by-count":0,"title":["Epithelioid inflammatory myofibroblastic uterine sarcoma"],"prefix":"10.17116","volume":"88","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-8242-7316","authenticated-orcid":false,"given":"I.A.","family":"Tarasov","sequence":"first","affiliation":[{"name":"N.N. 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