{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T18:43:01Z","timestamp":1793385781997,"version":"4.5.0"},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100018935","name":"Universit\u00e4tsklinikum Erlangen","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100018935","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Virchows Arch"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Angiomatoid fibrous histiocytoma (AFH) is a rare mesenchymal neoplasm of borderline malignancy (locally recurring, rarely metastasizing), most often involving the limbs, trunk, and head\/neck. Rarely, AFH may involve unusual locations. Herein, we characterize the clinicopathologic features of 26 AFH of the distal extremities, including acral sites. The tumors occurred in 19 females and 7 males ranging in age from 12 to 76\u00a0years (median, 23\u00a0years). Tumors involved the upper (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200919) and lower (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20096) distal extremity; one affected an unspecified digital site. Twenty-two cases occurred in acral locations (hands and feet). Subsets of cases showed the following morphologic features: multinodular architecture (26\/26), lymphoid cuffs (23\/26), prominent stromal myxoid change (11\/25), angiomatoid features (9\/26), and cytologic pleomorphism (8\/26). The average mitotic count was 1\/10 HPF; 3 cases showed brisk mitotic activity (&gt;\u200910 mitoses\/10 HPF). Immunohistochemistry revealed variable expression of desmin (16\/25), EMA (14\/21) and ALK (5\/8). Molecular testing revealed\n                    <jats:italic>EWSR1<\/jats:italic>\n                    rearrangements in 17\/18 cases (94%). Among 12 tumors with known fusion partners, the fusions partner was\n                    <jats:italic>CREB1<\/jats:italic>\n                    in 6 cases (50%),\n                    <jats:italic>CREM<\/jats:italic>\n                    in 4 tumors (33%),\n                    <jats:italic>ATF1<\/jats:italic>\n                    in one tumor (8%) and\n                    <jats:italic>PBX3<\/jats:italic>\n                    (8%) in another tumor. Prominent myxoid features were noted in 75%\n                    <jats:italic>CREM<\/jats:italic>\n                    versus 33% of\n                    <jats:italic>CREB1<\/jats:italic>\n                    versus 0% of\n                    <jats:italic>ATF1<\/jats:italic>\n                    -fused tumors. AFH occurring in distal extremity\/acral locations have a predilection for females, upper extremity locations, frequent unusual (solid, non-angiomatoid and myxoid) morphology and higher frequency of\n                    <jats:italic>CREM<\/jats:italic>\n                    over\n                    <jats:italic>ATF1<\/jats:italic>\n                    fusions. Awareness of the morphologic spectrum of these rare neoplasms is essential for correct classification.\n                  <\/jats:p>","DOI":"10.1007\/s00428-025-04199-y","type":"journal-article","created":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T04:52:02Z","timestamp":1754023922000},"page":"587-595","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Angiomatoid fibrous histiocytoma occurring at distal\/acral extremity sites: clinicopathological and molecular study of 26 cases highlighting frequent myxoid histology and site-dependent genotypic variation"],"prefix":"10.1007","volume":"487","author":[{"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy","family":"Molligan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatimah I.","family":"Alruwaii","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina R.","family":"Antonescu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth G.","family":"Demicco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brendan C.","family":"Dickson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Gross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Michal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle","family":"Perry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"T\u00f6gel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nasir Ud","family":"Din","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Folpe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,1]]},"reference":[{"key":"4199_CR1","doi-asserted-by":"publisher","first-page":"2147","DOI":"10.1002\/1097-0142(197912)44:6<2147::AID-CNCR2820440627>3.0.CO;2-8","volume":"44","author":"FM Enzinger","year":"1979","unstructured":"Enzinger FM (1979) Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. 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The study has been approved by the institutional review boards of contributing authors.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"The authors have no financial or non-financial conflicts of interest to disclose. AA is Editor-in-Chief of Virchows Archiv. ALF, BCD, CRA, JG & MM serve on the editorial board of Virchows Archiv.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}