{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T21:21:58Z","timestamp":1785878518091,"version":"3.56.0"},"reference-count":46,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,6,13]],"date-time":"2024-06-13T00:00:00Z","timestamp":1718236800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>The majority of dermatofibrosarcoma protuberans (DFSP) harbour <jats:italic>PDGFB<\/jats:italic> or <jats:italic>PDGFD<\/jats:italic> rearrangements. We encountered ALK expression\/rearrangement in a <jats:italic>PDGFB\/D<\/jats:italic>\u2010negative CD34\u2010positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of <jats:italic>ALK<\/jats:italic>\u2010rearrangements in DFSP and plaque\u2010like CD34\u2010positive dermal fibroma (P\u2010LDF).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and Results<\/jats:title><jats:p>We searched the archives of academic institutions for cases previously coded as DFSP and P\u2010LDF. NGS\u2010na\u00efve or <jats:italic>PDGFB<\/jats:italic>\u2010negative DFSP were screened for ALK (clone D5F3) expression by immunohistochemistry. NGS or <jats:italic>ALK<\/jats:italic> FISH was performed on ALK\u2010positive cases. Methylome profiling studies were performed and compared with conventional DFSP. One case of \u201cDFSP\u201d and two \u201cP\u2010LDF\u201d with ALK expression were identified from the archives, while four cases were detected prospectively. These seven cases (6F:1M; 8\u2009months to 76\u2009years) arose in the dermis of the arm (two), scalp, eyelid, thigh, abdomen, and shoulder and ranged from 0.4 to 4.2\u2009cm. Tumours were composed of spindled cells and displayed a storiform growth pattern. Cytologic atypia was absent, and mitotic figures were scarce (0\u20132\/10 HPFs, high power fields). The lesional cells were diffusely positive for CD34 and ALK and negative for S100 protein. By NGS (<jats:italic>n<\/jats:italic>\u2009=\u20095), <jats:italic>ALK<\/jats:italic> fusion partners included <jats:italic>DCTN1<\/jats:italic> (2), <jats:italic>PLEKHH2<\/jats:italic>, and <jats:italic>CLIP2<\/jats:italic> in DFSP\u2010like cases and <jats:italic>FLNA<\/jats:italic> in P\u2010LDF\u2010like lesions. <jats:italic>ALK<\/jats:italic> FISH was positive in one (of two) cases previously labelled P\u2010LDF. Methylome profiling of two (of three) <jats:italic>ALK<\/jats:italic>\u2010rearranged DFSP\u2010like tumours showed clustering with conventional DFSP in the UMAP dimension reduction plot. To date, no tumour has recurred (<jats:italic>n<\/jats:italic>\u2009=\u20092; 26, 27\u2009months).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We describe a cohort of novel <jats:italic>ALK<\/jats:italic>\u2010rearranged tumours with morphologic features similar to DFSP.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.15239","type":"journal-article","created":{"date-parts":[[2024,6,13]],"date-time":"2024-06-13T07:10:20Z","timestamp":1718262620000},"page":"649-659","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["<i>ALK<\/i>\u2010rearranged, <scp>CD34<\/scp>\u2010positive spindle cell neoplasms resembling dermatofibrosarcoma protuberans: a study of seven cases"],"prefix":"10.1111","volume":"85","author":[{"given":"Shruti","family":"Agrawal","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester MN USA"},{"name":"Department of 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Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Creytens","sequence":"additional","affiliation":[{"name":"Department of Pathology Ghent University and Ghent University Hospital  Ghent Belgium"},{"name":"CRIG Cancer Research Institute Ghent, Ghent University and Ghent University Hospital  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Sukov","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Baumhoer","sequence":"additional","affiliation":[{"name":"Bone Tumor Reference Center at the Institute for Medical Genetics and Pathology University Hospital Basel, University of Basel  Basel Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2278-5908","authenticated-orcid":false,"given":"Steven 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