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Both tumors typically manifest as nonspecific, often ulcerated, skin\u2010 to flesh\u2010colored nodules in chronically sun\u2010damaged skin of elderly male patients. AFX is a rather well demarcated, often rapidly growing tumor. PDS tumors are poorly circumscribed and are characterized by aggressive infiltrative growth. Fast as well as slow growth behavior has been reported for both tumors. Histologically, both are composed of spindle\u2010shaped and epithelioid tumor cells with pleomorphic nuclei as well as atypical multinucleated giant cells. Atypical mitoses are common. In contrast to AFX, PDS involves relevant parts of the subcutis and shows areas of tumor necrosis and\/or perineural infiltration. Due to the poorly differentiated nature of AFX\/PDS (Grade\u00a03), histopathologically similar cutaneous sarcomas, undifferentiated carcinomas, melanomas and other diseases have to be excluded by immunohistochemical analysis.<\/jats:p><jats:p>The treatment of choice is micrographically controlled surgery. In cases of AFX, a cure can be assumed after complete excision. Local recurrence rates are low as long as PDS tumors are surgically removed with a safety margin of 2\u00a0cm. Metastasis is rare and mostly associated with very thick or incompletely excised tumors; it mainly affects the skin and lymph nodes. Distant metastasis is even more rare. No approved and effective systemic therapy has been established.<\/jats:p>","DOI":"10.1111\/ddg.14700","type":"journal-article","created":{"date-parts":[[2022,1,31]],"date-time":"2022-01-31T12:32:43Z","timestamp":1643632363000},"page":"235-243","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["S1\u2010guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS)"],"prefix":"10.1111","volume":"20","author":[{"given":"Doris","family":"Helbig","sequence":"first","affiliation":[{"name":"Department of Dermatology and Venereology University Hospital Cologne  Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirjana","family":"Ziemer","sequence":"additional","affiliation":[{"name":"Department of Dermatology Venereology and Allergology University Hospital Leipzig  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