Abstract
Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of a disease spectrum. Both tumors typically manifest as nonspecific, often ulcerated, skin- to flesh-colored nodules in chronically sun-damaged 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-shaped 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 3), histopathologically similar cutaneous sarcomas, undifferentiated carcinomas, melanomas and other diseases have to be excluded by immunohistochemical analysis. 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 cm. 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.
| Original language | English |
|---|---|
| Journal | JDDG - Journal of the German Society of Dermatology |
| Volume | 20 |
| Issue number | 2 |
| Pages (from-to) | 235-243 |
| Number of pages | 9 |
| ISSN | 1610-0379 |
| DOIs | |
| Publication status | Published - 02.2022 |
Funding
Mirjana Ziemer has received consulting and lecturing fees as well as financial support for attending medical conventions from Bristol‐Myers Squibb, MSD Sharp & Dohme GmbH, Pfizer Pharma GmbH, and Sanofi‐Aventis Deutschland GmbH. Uwe Hillen has received remuneration for advisory board activities as well as lectures from the following companies: AbbVie, ALK‐Abelló, Almirall, Novartis, Sanofi, Takeda – none of them associated with the contents of this guideline. Ulrike Leiter has received remunerations for advisory board activities as well as lectures from the following companies: MSD, Novartis, Sun Pharma, Sanofi, Roche not associated with this publication, and research support from MSD. Selma Ugurel has received remunerations for research, lecturing, and advisory board activities as well as partial reimbursement for travel costs from Bristol Myers Squibb, Merck Sharp & Dohme, Merck Serono, Novartis and Roche. Jochen Utikal has received remuneration for advisory board activities and lectures as well as travel allowances from the following companies: Amgen, BMS, GSK, Leo Pharma, MSD, Novartis, Pierre Fabre, Sanofi, Roche, not associated with this publication. Doris Helbig, Edgar Dippel, Michael Erdmann, Thomas Mentzel, Georg Osterhoff, Dagmar von Bubnoff, Carsten Weishaupt, and Stephan Grabbe declare no conflicts of interest.
Research Areas and Centers
- Research Area: Luebeck Integrated Oncology Network (LION)
