{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T12:40:21Z","timestamp":1765370421546,"version":"build-2065373602"},"reference-count":0,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T00:00:00Z","timestamp":1716336000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background: Clear cell sarcoma (CCS) is an extremely rare form of sarcoma representing less than 1% of all soft-tissue sarcomas. It has morphological, structural, and immunohistochemical similarities to malignant melanoma, affecting young adults and equally affecting both sexes, and is usually located in the tendinous sheaths and aponeuroses of the limbs. Gastrointestinal localization is exceptional, with less than 100 cases reported thus far. The gene fusion of activating transcription factor 1 (ATF1) and the Ewing sarcoma breakpoint region 1 (EWSR1) are pathognomonic for clear cell sarcoma, representing the key to the diagnosis. CCS is an extremely aggressive tumor, with &gt;30% having distant or lymphatic metastasis at the time of diagnostic, and it has a high recurrence rate of over 80% in the first year after diagnosis and a high tendency for metastatic dissemination. Given the rarity of this tumor, there is no standardized treatment. Early diagnosis and radical surgery are essential in the treatment of CCS both for the primary tumor and for recurrence or metastasis. Chemo-radiotherapy has very little effect and is rarely indicated, and the role of targeted therapies is still under investigation. Case presentation: We present an extremely rare case of intestinal CSS in a 44-year-old Caucasian female. The patient, asymptomatic, first presented for a routine checkup and was diagnosed with mild iron-deficiency anemia. Given her family history of multiple digestive cancers, additional investigations were requested (gastroscopy, colonoscopy, tumoral markers and imaging) and the results were all within normal limits. In the subsequent period, the patient experienced mild diffuse recurrent abdominal pain, which occurred every 2\u20133 months. Two years later, the patient presented with symptoms of intestinal obstruction and underwent an emergency laparotomy followed by segmental enterectomy and regional lymphadenectomy for stenotic tumor of the jejunum. Histology, immunohistochemistry, and genetic testing established the diagnosis of CCS. No adjuvant therapy was indicated. Initially, no signs of recurrence or metastasis were detected, but after 30 and 46 months, respectively, from the primary treatment, the patient developed liver metastasis and pericolic peritoneal implants treated by atypical hepatic resections and right hemicolectomy. The patient remains under observation.<\/jats:p>","DOI":"10.3390\/medicina60060847","type":"journal-article","created":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T11:03:47Z","timestamp":1716375827000},"page":"847","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Intestinal Clear Cell Sarcoma\u2014A Case Presentation of an Extremely Rare Tumor and Literature Review"],"prefix":"10.3390","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6161-1951","authenticated-orcid":false,"given":"Vlad","family":"Rotaru","sequence":"first","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9225-8030","authenticated-orcid":false,"given":"Elena","family":"Chitoran","sequence":"additional","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Madalina Nicoleta","family":"Mitroiu","sequence":"additional","affiliation":[{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0839-0651","authenticated-orcid":false,"given":"Sinziana Octavia","family":"Ionescu","sequence":"additional","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ariana","family":"Neicu","sequence":"additional","affiliation":[{"name":"Pathology Department, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ciprian","family":"Cirimbei","sequence":"additional","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mihnea","family":"Alecu","sequence":"additional","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aisa","family":"Gelal","sequence":"additional","affiliation":[{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andra Delia","family":"Prie","sequence":"additional","affiliation":[{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurentiu","family":"Simion","sequence":"additional","affiliation":[{"name":"General Surgery Department 10, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 050474 Bucharest, Romania"},{"name":"General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology \u201cProf. Dr. Al. Trestioreanu\u201d, 022328 Bucharest, Romania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,5,22]]},"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/6\/847\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:46:47Z","timestamp":1760107607000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/6\/847"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,22]]},"references-count":0,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2024,6]]}},"alternative-id":["medicina60060847"],"URL":"https:\/\/doi.org\/10.3390\/medicina60060847","relation":{},"ISSN":["1648-9144"],"issn-type":[{"type":"electronic","value":"1648-9144"}],"subject":[],"published":{"date-parts":[[2024,5,22]]}}}