{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T12:48:20Z","timestamp":1782564500343,"version":"3.54.5"},"reference-count":51,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,7,5]],"date-time":"2021-07-05T00:00:00Z","timestamp":1625443200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Complete loss of SMARCB1\/INI1 in soft\u2010tissue tumours such as malignant rhabdoid tumour, epithelioid sarcoma, myoepithelial tumour of soft tissue and extraskeletal myxoid chondrosarcoma is often associated with high\u2010grade malignancy and poor prognosis. The diagnosis is sometimes challenging, owing to histological similarities, so careful differential diagnosis is required. Therefore, soft\u2010tissue tumours with complete SMARCB1\/INI1 loss could potentially include an unknown entity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>We analysed 160 cases of SMARCB1\/INI1\u2010deficient soft\u2010tissue tumour, and found 14 cases that were not classifiable into already existing categories and had common clinical and histological features. These involved two male and 12 female patients, ranging in age from 20\u00a0years to 61\u00a0years. The tumours were located in the the puboinguinal region (<jats:italic>n<\/jats:italic>\u00a0=\u00a013) and pelvic cavity (<jats:italic>n<\/jats:italic>\u00a0=\u00a01). Histologically, the tumours showed relatively uniform epithelioid to spindle\u2010shaped cells with myxoid stroma. All tumours showed immunoreactivity for brachyury, epithelial membrane antigen, and progesterone receptor, and 12 of 14 cases did so for oestrogen receptor. Variable positive staining for \u03b1\u2010smooth muscle actin, S100 and glial fibrillary acidic protein (GFAP) was seen. <jats:italic>NR4A3<\/jats:italic> and <jats:italic>EWSR1<\/jats:italic> gene rearrangements were not detected in 13 and 11 examined cases, respectively. Clinical follow\u2010up data for the 14 patients showed that 13 were alive without disease and one had been lost to follow\u2010up; four patients developed local recurrence and\/or metastases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The designation \u2018myxoepithelioid tumour with choroid features\u2019 (METC) was proposed as a tumour with intermediate malignancy controllable with appropriate treatment, including the entity of myoepithelioma\u2010like tumour of the vulvar region. METC represents a novel and independent subset that is histologically, biologically and clinically distinct from already existing SMARCB1\/INI1\u2010deficient soft\u2010tissue tumours.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.14393","type":"journal-article","created":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T09:25:04Z","timestamp":1619861104000},"page":"629-641","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Myxoepithelioid tumour with chordoid features: a clinicopathological, immunohistochemical and genetic study of 14 cases of SMARCB1\/INI1\u2010deficient soft\u2010tissue neoplasm"],"prefix":"10.1111","volume":"79","author":[{"given":"Izumi","family":"Kinoshita","sequence":"first","affiliation":[{"name":"Department of Anatomical Pathology Graduate School of Medical Sciences Kyushu University Fukuoka 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