{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T17:32:37Z","timestamp":1782495157003,"version":"3.54.5"},"reference-count":17,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Oncology"],"published-print":{"date-parts":[[2016]]},"abstract":"Malignant gastrointestinal neuroectodermal tumor (GNET) is an aggressive rare tumor, primarily occurring in young adults with frequent local-regional metastases and recurrence after local control. The tumor is characterized by the presence of <i>EWSR1-ATF1<\/i> or <i>EWSR1-CREB1<\/i> and immunohistochemical positivity for S-100 protein without melanocytic marker positivity. Due to poor responses to standard sarcoma regimens, GNET has a poor prognosis, and development of effective systemic therapy is desperately needed to treat these patients. Herein, we present a patient with a small bowel GNET who experienced recurrent hepatic and skeletal metastases after a primary resection. Comprehensive genomic profiling (CGP) in the course of clinical care with DNA and RNA sequencing demonstrated the presence of an exon 7 to exon 6 <i>EWSR1-CREB1<\/i> fusion in the context of a diploid genome with no other genomic alterations. In a clinical trial, the patient received a combination of 250 mg crizotinib with 600 mg pazopanib quaque die and achieved partial response and durable clinical benefit for over 2.8 years, and with minimal toxicity from therapy. Using a CGP database of over 50,000 samples, we identified 11 additional cases that harbor <i>EWSR1-CREB1<\/i> and report clinicopathologic characteristics, as these patients may also benefit from such a regimen.<\/jats:p>","DOI":"10.1159\/000449204","type":"journal-article","created":{"date-parts":[[2016,10,20]],"date-time":"2016-10-20T21:02:08Z","timestamp":1476997328000},"page":"348-353","source":"Crossref","is-referenced-by-count":39,"title":["Activity of c-Met\/ALK Inhibitor Crizotinib and Multi-Kinase VEGF Inhibitor Pazopanib in Metastatic Gastrointestinal Neuroectodermal Tumor Harboring EWSR1-CREB1 Fusion"],"prefix":"10.1159","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6064-6837","authenticated-orcid":false,"given":"Vivek","family":"Subbiah","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Holmes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle","family":"Gowen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Spritz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Behrang","family":"Amini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-Lien","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexa 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M.","family":"Ali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2016,10,21]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1177\/106689690301100202"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31824644ac"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000404"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(196509)18:9<1163::AID-CNCR2820180916>3.0.CO;2-0"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-012-1295-y"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.2696"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1311107"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1126\/science.1099314"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2015.09.006"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2015.02.005"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-06-1570"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0710370104"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2014.03.013"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-09-1121"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1186\/s13045-015-0160-2"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoncol.2015.5689"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000364"}],"container-title":["Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/449204","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/449204","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/449204","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T17:10:22Z","timestamp":1745428222000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000449204"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016]]},"references-count":17,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2016,11,30]]}},"URL":"https:\/\/doi.org\/10.1159\/000449204","archive":["Portico"],"relation":{},"ISSN":["0030-2414","1423-0232"],"issn-type":[{"value":"0030-2414","type":"print"},{"value":"1423-0232","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016]]}}}