Ugurel, Selma and Abu Rached, Nessr and Gambichler, Thilo and Mengoni, Miriam and Tueting, Thomas and Hassel, Jessica C. and Reschke, Robin and Lodde, Georg C. and Placke, Jan-Malte and Schadendorf, Dirk and Reinhardt, Lydia and Kreuter, Alexander and Gschnell, Martin and Utikal, Jochen and Erdmann, Michael and Duecker, Pia and Helbig, Doris and Franklin, Cindy and Gebhardt, Christoffer and Poch, Gabriela and Kaehler, Katharina C. and Weichenthal, Michael and Gutzmer, Ralf and Heinzerling, Lucie and Weishaupt, Carsten and Mohr, Peter and Thoms, Kai-Martin and Lang, Berenice and Schilling, Bastian and Haferkamp, Sebastian and Sachse, Michael and Welzel, Julia and Jochims, Franziska and Raap, Ulrike and Schley, Gaston and Terheyden, Patrick and Dippel, Edgar and Poettgen, Christoph and Becker, Jurgen C. and Tasdogan, Alpaslan and Amaral, Teresa and Nanz, Lena and Leiter, Ulrike (2025) Outcome of systemic therapy in patients with advanced rare skin cancers: A retrospective multicenter DeCOG study of 209 patients. EUROPEAN JOURNAL OF CANCER, 228: 115750. ISSN 0959-8049, 1879-0852
Full text not available from this repository. (Request a copy)Abstract
Introduction: For rare skin cancers, few data exist on the outcome of systemic therapies, particularly immune checkpoint inhibition (ICI). The present study analysed the real-world use of different systemic therapies including ICI, and its outcome in patients with advanced rare skin cancers.<br /> Methods: This retrospective multicenter study included patients who received systemic therapy for advanced, non-resectable cutaneous angiosarcoma (AS), Kaposi sarcoma (KS), pleomorphic dermal sarcoma (PDS), or cutaneous adnexal carcinoma (CAC). Study endpoints were best overall response (BOR), progression-free survival (PFS) and overall survival (OS).<br /> Results: 209 patients (77 AS; 81 KS; 14 PDS; and 37 CAC) from 30 centers were included. As first-line treatment AS and KS patients predominantly received chemotherapy (77.9 %; 63.0 %), while PDS and CAC patients mostly received ICI (64.4 %; 43.2 %). BOR in first-line across all therapy types was 65.5 % in KS, 50.0 % in PDS, 41.6 % in AS, and 10.8 % in CAC. BOR for ICI was 66.6 % for PDS, 58.3 % for AS, 33.3 % for KS, and 4.3 % for CAC, irrespective of treatment line. 1-year PFS rate upon any first-line therapy was 70.7 % for PDS, 45.7 % for KS, 25.6 % for AS, and 18.5 % for CAC (p < 0.001). 1-year tumor-specific OS rate was 97.3 % in KS, 84.2 % in AS, 67.7 % in PDS, and 65.4 % in CAC (p < 0.001).<br /> Conclusions: Type and outcome of systemic therapy differed between cancer entities. Efficacy of ICI was high in PDS and AS, moderate in KS, and low in CAC. Patients with advanced CAC revealed an extremely poor prognosis regardless of the type of therapy used.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ADENOID CYSTIC CARCINOMA; SINGLE-ARM; PEMBROLIZUMAB; METASTASIS; SARCOMA; PHASE-2; Angiosarcoma; Kaposi sarcoma; Cutaneous adnexal carcinoma; Pleomorphic dermal sarcoma; Immune checkpoint inhibition; Chemotherapy; Targeted therapy |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Dermatologie und Venerologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 24 Mar 2026 13:54 |
| Last Modified: | 24 Mar 2026 13:54 |
| URI: | https://pred.uni-regensburg.de/id/eprint/68043 |
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