{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T08:38:01Z","timestamp":1792658281183,"version":"4.5.0"},"reference-count":86,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T00:00:00Z","timestamp":1792627200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T00:00:00Z","timestamp":1792627200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Deutsche Derma Gesell"],"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of one disease spectrum. Both tumors manifest as nonspecific, often ulcerated nodules in chronically sun\u2010damaged skin. AFX is a well\u2010demarcated, often rapidly growing tumor. PDS tumors are poorly circumscribed and characterized by a more aggressive infiltrative growth pattern. Histologically, both are composed of spindle\u2010shaped and epithelioid tumor cells with pleomorphic nuclei as well as atypical multinucleated tumor giant cells. Atypical mitoses are common. In contrast to AFX, PDS extends into the subcutis and shows areas of tumor necrosis and\/or perineural infiltration. Due to the undifferentiated morphology of AFX\/PDS, histopathologically similar tumors must be excluded by immunohistochemical analysis.<\/jats:p>\n                  <jats:p>The treatment of choice is microscopically controlled surgery. In cases of AFX, cure can be assumed after complete excision. When PDS tumors are surgically excised with a 2 cm margin or by microscopically controlled surgery, local recurrence rates are low. Metastases are rare and are mostly associated with very thick or incompletely excised tumors, primarily affecting the skin and lymph nodes. Distant metastases are even rarer. There is currently no approved and effective systemic therapy. Treatment with immune checkpoint inhibitors appears promising.<\/jats:p>","DOI":"10.1111\/ddg.70471x","type":"journal-article","created":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T07:40:11Z","timestamp":1792654811000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Update of the S1\u2010guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS)"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5841-4631","authenticated-orcid":false,"given":"Doris","family":"Helbig","sequence":"first","affiliation":[{"name":"Department of Dermatology and Venereology University Hospital Cologne  Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6488-1917","authenticated-orcid":false,"given":"Mirjana","family":"Ziemer","sequence":"additional","affiliation":[{"name":"Department of Dermatology Venereology and Allergology University Hospital Leipzig  Leipzig Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7479-5454","authenticated-orcid":false,"given":"Diana","family":"Crisan","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology University Hospital Ulm  Ulm Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4433-2387","authenticated-orcid":false,"given":"Edgar","family":"Dippel","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Ludwigshafen City Hospital  Ludwigshafen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7136-6489","authenticated-orcid":false,"given":"Michael","family":"Erdmann","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital Erlangen CCC Erlangen EMN  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8019-1577","authenticated-orcid":false,"given":"Uwe","family":"Hillen","sequence":"additional","affiliation":[{"name":"MVZ Dermatopathology Duisburg\u2010Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muemtaz","family":"Koeksal","sequence":"additional","affiliation":[{"name":"Department of Radiation Therapy and Radiation Oncology Hospital Bielefeld\u2010Mitte Medical Faculty and University Hospital OWL Bielefeld University  Bielefeld Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"Leiter","sequence":"additional","affiliation":[{"name":"Center for Dermato\u2010Oncology University Hospital Dermatology Eberhard Karls University T\u00fcbingen  T\u00fcbingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Mentzel","sequence":"additional","affiliation":[{"name":"Kressbronn am Bodensee, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars Erik","family":"Podleska","sequence":"additional","affiliation":[{"name":"Department of Tumor Orthopedics and Sarcoma Surgery University Hospital Essen  Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Utikal","sequence":"additional","affiliation":[{"name":"Clinical Cooperation Unit Dermato\u2010Oncology of the German Cancer Research Center Heidelberg (DKFZ) and Department of Dermatology Venereology and Allergology Medical Faculty Mannheim Ruprecht Karls University Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5510-9095","authenticated-orcid":false,"given":"Dagmar","family":"von Bubnoff","sequence":"additional","affiliation":[{"name":"Department of Dermatology Allergology and Venereology University Hospital Schleswig\u2010Holstein  Campus L\u00fcbeck 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