{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:57:47Z","timestamp":1760245067006,"version":"3.44.0"},"reference-count":19,"publisher":"BMJ","issue":"8","license":[{"start":{"date-parts":[[2021,8,26]],"date-time":"2021-08-26T00:00:00Z","timestamp":1629936000000},"content-version":"unspecified","delay-in-days":25,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,8,15]]},"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:p>A 55-year-old woman presented with a 3-month history of right groin swelling, discomfort and impaired mobility. On examination, a palpable mass was noted both to the right of midline in the lower abdomen and in the right groin. MRI of the pelvis showed two masses involving the anterior abdominal wall and right groin, as well as lymph node involvement. CT imaging revealed multiple bilateral pulmonary metastases. Pathology demonstrated a myxohayline stroma morphology. Tumour was also notable for <jats:italic>NR4A3<\/jats:italic> gene region rearrangement and mutation in <jats:italic>KIT<\/jats:italic> exon 11 at position c.1669 T&gt;G. Based on these findings, she was diagnosed with extraskeletal myxoid chondrosarcoma (EMC). The patient has been on imatinib, a tyrosine kinase inhibitor with activity against KIT, for 3 years with stable disease. Metastatic EMC is generally treated with surgical resection and perioperative radiation therapy with adjuvant chemotherapy and is associated with poor prognosis.<\/jats:p>","DOI":"10.1136\/bcr-2021-242039","type":"journal-article","created":{"date-parts":[[2021,8,26]],"date-time":"2021-08-26T12:45:15Z","timestamp":1629981915000},"page":"e242039","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Sustained response to imatinib in patient with extraskeletal myxoid chondrosarcoma and novel <i>KIT<\/i> mutation"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4751-7288","authenticated-orcid":false,"given":"Brooke","family":"Jennings","sequence":"first","affiliation":[{"name":"Department of Molecular Physiology and Biophysics, The University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USA"},{"name":"Department of Internal Medicine, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Rieth","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA"},{"name":"Holden Comprehensive Cancer Center, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Travis","family":"Snyders","sequence":"additional","affiliation":[{"name":"Holden Comprehensive Cancer Center, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammed","family":"Milhem","sequence":"additional","affiliation":[{"name":"Holden Comprehensive Cancer Center, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,8,26]]},"reference":[{"key":"2025082802080972000_14.8.e242039.1","article-title":"Unresectable extraskeletal myxoid chondrosarcoma of the neck: early tumor response to chemoradiotherapy","volume":"7","author":"Zaki","year":"2015","journal-title":"Cureus"},{"key":"2025082802080972000_14.8.e242039.2","first-page":"1","article-title":"Extraskeletal myxoid chondrosarcoma: a comparative study of imaging and pathology","volume":"2018","author":"Zhang","year":"2018","journal-title":"Biomed Res Int"},{"key":"2025082802080972000_14.8.e242039.3","doi-asserted-by":"publisher","DOI":"10.1158\/1055-9965.EPI-20-0447"},{"key":"2025082802080972000_14.8.e242039.4","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-012-1240-0"},{"key":"2025082802080972000_14.8.e242039.5","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.jmoldx.2013.12.002","article-title":"Diagnostic utility of molecular investigation in extraskeletal myxoid chondrosarcoma","volume":"16","author":"Benini","year":"2014","journal-title":"J Mol Diagn"},{"key":"2025082802080972000_14.8.e242039.6","doi-asserted-by":"crossref","first-page":"21770","DOI":"10.18632\/oncotarget.15568","article-title":"Next generation sequencing of extraskeletal myxoid chondrosarcoma","volume":"8","author":"Davis","year":"2017","journal-title":"Oncotarget"},{"key":"2025082802080972000_14.8.e242039.7","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20044"},{"key":"2025082802080972000_14.8.e242039.8","doi-asserted-by":"crossref","DOI":"10.3390\/ijms19071855","article-title":"Identification of an actionable mutation of kit in a case of extraskeletal myxoid chondrosarcoma","volume":"19","author":"Urbini","year":"2018","journal-title":"Int J Mol Sci"},{"key":"2025082802080972000_14.8.e242039.9","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)09292-9"},{"key":"2025082802080972000_14.8.e242039.10","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-198209000-00009"},{"key":"2025082802080972000_14.8.e242039.11","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.23978"},{"key":"2025082802080972000_14.8.e242039.12","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2014.03.013"},{"key":"2025082802080972000_14.8.e242039.13","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1200\/JCO.1987.5.6.840","article-title":"Randomized comparison of three adriamycin regimens for metastatic soft tissue sarcomas","volume":"5","author":"Borden","year":"1987","journal-title":"J Clin Oncol"},{"key":"2025082802080972000_14.8.e242039.14","doi-asserted-by":"publisher","DOI":"10.1016\/S0016-5085(98)70079-4"},{"key":"2025082802080972000_14.8.e242039.15","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2006.09.001"},{"key":"2025082802080972000_14.8.e242039.16","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1097\/CMR.0000000000000454","article-title":"The clinical significance of kit mutations in melanoma: a meta-analysis","volume":"28","author":"Gong","year":"2018","journal-title":"Melanoma Res"},{"key":"2025082802080972000_14.8.e242039.17","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.hemonc.2016.08.005","article-title":"Prognostic importance of c-kit mutations in core binding factor acute myeloid leukemia: a systematic review","volume":"10","author":"Ayatollahi","year":"2017","journal-title":"Hematol Oncol Stem Cell Ther"},{"key":"2025082802080972000_14.8.e242039.18","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2007.13.4403"},{"key":"2025082802080972000_14.8.e242039.19","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1609324"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2021-242039","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T09:08:17Z","timestamp":1756372097000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2021-242039"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8]]},"references-count":19,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2021,8,26]]},"published-print":{"date-parts":[[2021,8]]}},"alternative-id":["10.1136\/bcr-2021-242039"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2021-242039","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2021,8]]}}}