{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T07:05:20Z","timestamp":1792479920056,"version":"4.5.0"},"reference-count":58,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,12,28]],"date-time":"2023-12-28T00:00:00Z","timestamp":1703721600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm of intermediate malignant potential, occurring at any age and at multiple sites. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of IMT, typically involving the abdomen. Most IMTs harbour kinase gene fusions, especially involving <jats:italic>ALK<\/jats:italic> and <jats:italic>ROS1<\/jats:italic>, but 20\u201330% of IMTs show no detectable translocations. The aim of this study is to further delineate clinicopathological and molecular characteristics of abdominal IMT and discover potential new therapeutic targets.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>In 20 IMTs, including four EIMS, RNA fusion analysis was performed, followed by multiplex DNA analysis if no <jats:italic>ALK<\/jats:italic> or <jats:italic>ROS1<\/jats:italic> fusion was detected. Fourteen IMTs (70.0%) had an <jats:italic>ALK<\/jats:italic> translocation and the fusion partner was identified in 11, including a <jats:italic>RRBP1::ALK<\/jats:italic> fusion, not previously described in classical (non\u2010EIMS) IMT. <jats:italic>RANBP2::ALK<\/jats:italic> fusion was demonstrated in all EIMS. One IMT had a <jats:italic>ROS1<\/jats:italic> fusion. In all <jats:italic>ALK\/ROS1<\/jats:italic> translocation\u2010negative IMTs mutations or fusions \u2013 as yet unreported in primary IMT \u2013 were found in genes related to the receptor tyrosine kinase (RTK)\/PI3K\/AKT pathway. Three of four patients with EIMS died of disease [mean survival 8\u2009months (4\u201315\u2009months)], whereas only one of 14 classical IMT patients succumbed to disease [mean follow\u2010up time 52\u2009months (2\u2013204 months); <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01].<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study shows the wide clinical spectrum of abdominal IMTs and affirms the poor prognosis of EIMS, raising discussion about its status as IMT subtype. Furthermore, the newly detected alterations of the RTK\/PI3K\/AKT pathway expand the molecular landscape of IMTs and provide potential therapeutic targets.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.15122","type":"journal-article","created":{"date-parts":[[2023,12,29]],"date-time":"2023-12-29T06:06:01Z","timestamp":1703829961000},"page":"794-809","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Abdominal inflammatory myofibroblastic tumour: Clinicopathological and molecular analysis of 20 cases, highlighting potential therapeutic targets"],"prefix":"10.1111","volume":"84","author":[{"given":"Astrid I P","family":"Vernemmen","sequence":"first","affiliation":[{"name":"Department of Pathology, School for Oncology and Reproduction (GROW) Maastricht University Medical Center  Maastricht The 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