{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T22:13:08Z","timestamp":1776982388340,"version":"3.51.4"},"reference-count":27,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:00:00Z","timestamp":1760140800000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    <jats:italic>EWSR1::CREM<\/jats:italic>\n                    fusion positive intrabdominal sarcomas defines a rare emerging group of aggressive mesenchymal neoplasms with a predilection for the celomic cavity and often manifesting perplexing immunophenotypic profile. There is no specific standard of care therapeutic option though anecdotal reports response to pazopanib have been reported. We report herein a case of a 62\u2010year\u2010old lady who presented with lower abdominal mass and pain. Right hemicolectomy performed showed a malignant epithelioid neoplasm. By immunohistochemistry, the tumor cells were positive for CD117, keratins, and mutation analysis was positive for\n                    <jats:italic>KIT<\/jats:italic>\n                    p.V5301 mutation, initially prompting a diagnosis of epithelioid gastrointestinal stromal tumor. However, the patient developed omental relapses while on imatinib and progressive disease persisted despite change to sunitinib. A re\u2010review of the histopathology of both the hemicolectomy and relapsed omental sites showed marked cellular atypia, increased cellularity and mitosis in metastatic omental sites. By immunohistochemistry, the tumors in addition to CD117 positivity were also immunoreactive to keratins, synaptophysin, chromogranin, CD56, and MUC4. Next generation sequencing performed on both the primary and relapsed sites were positive for\n                    <jats:italic>EWSR1::CREM<\/jats:italic>\n                    gene fusion in addition to the\n                    <jats:italic>KIT. P.V5301<\/jats:italic>\n                    mutation. The tumor was then reclassified as\n                    <jats:italic>EWSR1::CREM<\/jats:italic>\n                    fusion positive intrabdominal unclassified epithelioid sarcoma with concomitant KIT mutation of unknown significance. The patient was treated with cytotoxic chemotherapy and radiation therapy with partial response, but treatment was stopped because of complications and lack of tolerance. Pazopanib as a single agent was then initiated, and patients have continued to respond for the past 7\u2009months. We highlight the significant immunophenotypic heterogeneity in these tumors and perform an extensive review of the literature of this specific entity and broader group of\n                    <jats:italic>EWSR1::CREM\/FUS<\/jats:italic>\n                    fusion positive tumors ranging from quasi\u2010benign to malignant and finally we underscore the therapeutic utility of pazopanib as a single agent in this rare group of aggressive sarcomas.\n                  <\/jats:p>","DOI":"10.1002\/gcc.70062","type":"journal-article","created":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T16:03:46Z","timestamp":1760198626000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Durable Response to Pazopanib (Tyrosine Kinase Inhibitor) in a Patient With\n                    <i>EWSR1::CREM<\/i>\n                    Gene Fusion Positive Intra\u2010Abdominal Unclassified Epithelioid Sarcoma"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8468-2402","authenticated-orcid":false,"given":"Saba","family":"Shafi","sequence":"first","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4688-3780","authenticated-orcid":false,"given":"Dan","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine The Ohio State University Wexner Medical Center  Columbus Ohio USA"},{"name":"The James Comprehensive Cancer Center The Ohio State University  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaokang","family":"Pan","sequence":"additional","affiliation":[{"name":"James Molecular Laboratory The Ohio State University  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marium","family":"Husain","sequence":"additional","affiliation":[{"name":"Division of Medical Oncology, Department of Internal Medicine The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0908-2140","authenticated-orcid":false,"given":"Alan","family":"Rogers","sequence":"additional","affiliation":[{"name":"Department of Radiology The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5177-0652","authenticated-orcid":false,"given":"Steve","family":"Oghumu","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2012-1785","authenticated-orcid":false,"given":"O. Hans","family":"Iwenofu","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine The Ohio State University Wexner Medical Center  Columbus Ohio USA"},{"name":"The James Comprehensive Cancer Center The Ohio State University  Columbus Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,10,11]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/35085068"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(02)00828-0"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.68.1.821"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0303-7207(01)00696-7"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molmed.2013.02.001"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-05-2811"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20491"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20881"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-018-0953-z"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2011.08.004"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000864"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2018.10.007"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318227e4d2"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001331"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001114"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000788"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-020-0646-5"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.3390\/medicina60040544"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1215003"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmoldx.2023.11.004"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-021-03140-3"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1177\/1066896920961174"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/pin.13030"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncoscience.587"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(12)60651-5"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10456-009-9160-6"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2014.03.013"}],"container-title":["Genes, Chromosomes and Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/gcc.70062","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T13:43:56Z","timestamp":1761831836000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/gcc.70062"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10]]},"references-count":27,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2025,10]]}},"alternative-id":["10.1002\/gcc.70062"],"URL":"https:\/\/doi.org\/10.1002\/gcc.70062","archive":["Portico"],"relation":{},"ISSN":["1045-2257","1098-2264"],"issn-type":[{"value":"1045-2257","type":"print"},{"value":"1098-2264","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10]]},"assertion":[{"value":"2025-05-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-20","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70062"}}