{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T02:31:10Z","timestamp":1791858670579,"version":"4.3.4"},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T00:00:00Z","timestamp":1732752000000},"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":[[2025,4]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Epithelioid myxofibrosarcoma (eMFS) is an aggressive morphological variant associated with high rates of local recurrence and metastatic disease. The clinicopathological understanding of this disease is currently limited to a few small case\u2010series.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>We reviewed 44 cases of eMFS and classified them based on the presence of focal (&lt;\u200950%) or diffuse (&gt;\u200950%) epithelioid morphology and F\u00e9d\u00e9ration Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade. The patients (28 males; 16 females) had a median age of 71\u2009years (range\u2009=\u200914\u201390\u2009years). The proximal extremity was the most common site (<jats:italic>n<\/jats:italic>\u2009=\u200921), followed by the trunk (<jats:italic>n<\/jats:italic>\u2009=\u200911), distal extremity (<jats:italic>n<\/jats:italic>\u2009=\u2009nine) and head\/neck (<jats:italic>n<\/jats:italic>\u2009=\u2009two). Of cases with known depth of involvement (<jats:italic>n<\/jats:italic>\u2009=\u200941), 39 involved the subcutis, one was limited to the dermis and one limited to the skeletal muscle. Most cases (<jats:italic>n<\/jats:italic>\u2009=\u200934, 77%) demonstrated diffuse (&gt;\u200950%) epithelioid morphology and were FNCLCC grade 3 (<jats:italic>n<\/jats:italic>\u2009=\u200929, 66%). Follow\u2010up data were available for 22 patients. Two developed local recurrence and 10\u2009developed metastases, frequently to regional lymph nodes. All metastatic tumours had a primary lesion with diffuse epithelioid morphology (<jats:italic>P<\/jats:italic>\u2009=\u20090.09). There was no association between grade and recurrent or metastatic disease (<jats:italic>P<\/jats:italic>\u2009=\u20090.67 and 0.90, respectively). Three cases initially diagnosed as eMFS, one in the neck and two in the axilla, were found to have <jats:italic>NRAS<\/jats:italic> Q61R mutations and a high tumour mutation burden and\/or ultraviolet (UV)\u2010light DNA mutational signature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These findings suggest that UV\u2010driven malignancies (including melanoma or sarcomatoid squamous cell carcinoma) may histologically mimic eMFS and should be considered in cases of eMFS presenting at atypical anatomical sites.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.15373","type":"journal-article","created":{"date-parts":[[2024,11,29]],"date-time":"2024-11-29T05:23:40Z","timestamp":1732857820000},"page":"694-703","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Myxofibrosarcoma with epithelioid morphology: A clinicopathological study of 44 cases with emphasis on differential diagnosis"],"prefix":"10.1111","volume":"86","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3200-0166","authenticated-orcid":false,"given":"Alexandra L","family":"Isaacson","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan S","family":"Berry","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica","family":"Ulici","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan M","family":"Armstrong","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Bena","sequence":"additional","affiliation":[{"name":"Section of Biostatistics, Quantitative Health Sciences Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4751-7678","authenticated-orcid":false,"given":"Ivy","family":"John","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Pittsburgh Medical Center  Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arivarasan","family":"Karunamurthy","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Pittsburgh Medical Center  Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2278-5908","authenticated-orcid":false,"given":"Steven D","family":"Billings","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1139-2914","authenticated-orcid":false,"given":"Josephine K","family":"Dermawan","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Goldblum","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott E","family":"Kilpatrick","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Cleveland Clinic  Cleveland OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0657-3024","authenticated-orcid":false,"given":"Brian 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