Outcome of systemic therapy in patients with advanced rare skin cancers: A retrospective multicenter DeCOG study of 209 patients
- PMID: 40912056
- DOI: 10.1016/j.ejca.2025.115750
Outcome of systemic therapy in patients with advanced rare skin cancers: A retrospective multicenter DeCOG study of 209 patients
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.
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).
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).
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.
Keywords: Angiosarcoma; Chemotherapy; Cutaneous adnexal carcinoma; Immune checkpoint inhibition; Kaposi sarcoma; Pleomorphic dermal sarcoma; Targeted therapy.
Copyright © 2025 The Authors. Published by Elsevier Ltd.. All rights reserved.
Conflict of interest statement
Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Selma Ugurel: relevant financial activities (research support from Bristol Myers Squibb and Merck Serono; speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp and Dohme, Merck Serono, Novartis and Roche, and travel support from Bristol Myers Squibb, and Merck Sharp and Dohme). Nessr Abu Rached: received funding, travel support, and/or personal honoraria for lectures from Novartis Pharma, Janssen-Cilag GmbH and Johnson & Johnson that were independent of the work submitted. Thilo Gambichler: relevant financial activities (speakers and/or advisory board honoraria from BMS, Sanofi/Regeneron, MSD, Novartis Pharma, Roche, Abbvie, Almirall, UCB, Sun Pharmaceutical, Janssen, Lilly, Pfizer, Pierre Fabre, Merck-Serono). Miriam Mengoni: relevant financial activities (speakers or advisory board honoraria from Merck Sharp and Dohme, Novartis, Bristol-Myers Squibb, Sun Pharma, Pierre Fabre and Kyowa Kirin and travel support from Sun Pharma, Delcath and Pierre Fabre). Jessica C. Hassel: relevant financial activities (research support from Bristol Myers Squibb; speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp and Dohme, Novartis, Roche, Pierre Fabre and Sanofi Aventis, and travel support from Bristol Myers Squibb and Pierre Fabre). Robin Reschke: travel support from SunPharma. Georg C. Lodde received travel support from Sun Pharma, Pierre Fabre, research funding from Novartis. Jan-Malte Placke served as consultant and/or has received honoraria from Bristol-Myers Squibb, Novartis, Sanofi, MedKomAkademie and received travel support from Bristol-Myers Squibb, Novartis, Pierre Fabre and Therakos. Dirk Schadendorf: relevant financial activities (Roche, Novartis, Bristol-Myers Squibb, Merck Sharp & Dohme, Sanofi, Regeneron, Array, Pierre Fabre, 4SC, Helsinn, Philogen, InFlarX, Merck-Serono, SunPharma, Ultimovacs, Sandoz). Jochen Utikal is on the advisory board or has received honoraria and travel support from Amgen, Bristol Myers Squibb, GSK, Immunocore, LeoPharma, Merck Sharp and Dohme, Novartis, Pierre Fabre, Rheacell, Roche and Sanofi outside the submitted work Doris Helbig: relevant financial activities (Pierre Fabre and Sanofi-Aventis). Cindy Franklin: relevant financial activities (advisory board or has received honoraria from Bristol Myers Squibb, Immunocore and Novartis and received travel grants from Bristol Myers Squibb, Novartis and Pierre Fabre). Christoffer Gebhardt: relevant financial activities (advisory board or honoraria from Almirall, Amgen, Beiersdorf, BioNTech, Bristol-Myers Squibb, Immunocore, Janssen, MSD Sharp & Dohme, Novartis, Pierre-Fabre Pharma, Regeneron, Roche, Sanofi Genzyme, SUN Pharma and Sysmex, research funding from Bristol-Myers Squibb, Novartis, Regeneron and Sanofi, outside the submitted work, co-founder of Dermagnostix and Dermagnostix R&D). Gabriela Poch: relevant financial activities (speakers honoraria or travel support from MSD, BMS, Novartis, Sun Pharma, Pierre Fabre and Amgen). Katharina C. Kähler: relevant financial activities (Honoraria: BMS, MSD, Novartis, Sanofi-Aventis, Immunocore, Philogen; Consulting or Advisory Role: BMS, MSD, Pierre Fabre, Philogen, Sun Pharma; Research Funding: Novartis; Travel, Accommodations, Expenses: BMS, MSD, Novartis, Roche, Pierre Fabre, Sun Pharma). Michael Weichenthal: relevant financial activities (speakers and advisory board honoraria from Merck, Bristol-Myers Squibb, Roche, Novartis, Sanofi, Pierre Fabre, Beiersdorf, Sun Pharma, and Takeda). Ralf Gutzmer: grants (to institution) from Almirall Hermal, Amgen, Kyowa Kirin, Merck Serono, Recordati, Regeneron Pharmaceuticals, Sanofi, and Sun Pharmaceutical Industries; consulting fees from 4SC, Almirall Hermal, Bristol Myers Squibb, Delcath, Immunocore, Kyowa Kirin, Merck Serono, MSD, Novartis Pharmaceuticals, Pierre Fabre, Sanofi, and Sun Pharmaceutical Industries; honoraria from Almirall Hermal, Bristol Myers Squibb, Merck Serono, MSD, Novartis Pharmaceuticals, Pierre Fabre, Regeneron Pharmaceuticals, Sanofi, and Sun Pharmaceutical Industries; support for attending meetings and/or travel from Pierre Fabre and Sun Pharmaceutical Industries. Lucie Heinzerling: relevant financial activities (speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp and Dohme, Merck Serono, Novartis, Amgen, Curevac, Pierre Fabre, Sanofi and Roche). Peter Mohr: relevant financial activities (payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: MSD, Novartis, BMS, Pierre Fabre, Sanofi Genzyme, Immunocore, Regeneron, Delcath, Sun Pharma; participation on a Data Safety Monitoring Board or Advisory Board: MSD, Novartis, BMS, Pierre Fabre, Sanofi Genzyme, Immunocore, Regeneron, Delcath, Sun Pharma, Biotech; support for attending meetings and/or travel: MSD, BMS, Sun Pharma, Novartis). Kai-Martin Thoms: relevant financial activities (speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp & DOhme, Pierre Fabre, Novartis, Roche, Immunocore, Sanofi, Sun Pharma, Amgen, LEO, Galderma, Almirall, Candela, and Lilly). Berenice Lang: relevant financial activities (Pierre Fabre, Galderma, Sanofi, SUN Pharma, Novartis, Pfizer, Biofrontera, Almirall, Loreal). Bastian Schilling: relevant financial activities (speakers and advisory board honoraria from Sanofi, Pierre Fabre Pharmaceuticals, Regeneron, Immunocore and SUN Pharma). Sebastian Haferkamp: relevant financial activities (research support from Bristol Myers Squibb; speakers and advisory board honoraria from Bristol Myers Squibb, Merck Sharp and Dohme, Novartis, Sanofi and Roche). Julia Welzel: speakers and advisory board honoraria and travel support from Abbvie, Almirall, BMS, Boehringer Ingelheim, Janssen, Infectopharm, Leo, Lilly, Mibe, MSD, Novartis, Pfizer, Sanofi. Franziska Jochims: relevant financial activities (congress participation from Pierre-Fabre, UCB) Gaston Schley: relevant financial activities (BMS and Kiowa Kirin). Patrick Terheyden served as consultant and/or received honoraria form Almirall, Bristol Myers Squibb, Biofrontera, Kyowa Kirin, L’Oréal, Merck Sharp & Dohme, Novartis, Pierre-Fabre, Sanofi, 4SC, and travel support from Bristol Myers Squibb outside the submitted work. Jürgen C. Becker: is receiving speaker’s bureau honoraria from Amgen, MerckSerono, Pfizer, Sanofi and Sun Pharma and is a paid consultant/advisory board member/DSMB member for Almirall, Boehringer Ingelheim, ICON, Pfizer, Regeneron and Sanofi. His group receives research grants from Alcedis, Merck Serono/IQVIA, and Regeneron. Teresa Amaral: reports personal fees for advisory board membership from Delcath and Philogen; personal fees as an invited speaker from BMS, Neracare, Novartis and Pierre Fabre; personal fees for a writing engagement from CeCaVa and Medtrix; institutional fees as local principal investigator (PI) from Agenus Inc., AstraZeneca, BioNTech, BMS, HUYA Bioscience, Immunocore, IO Biotech, MSD, Pfizer, Philogen, Regeneron, Roche and University Hospital Essen; institutional fees as coordinating PI from Unicancer; institutional research grants from iFIT and Novartis; institutional funding from MNI - Naturwissenschaftliches und Medizinisches Institut, Neracare, Novartis, Pascoe, Sanofi and Skyline-Dx; non-remunerated membership of the American Society of Clinical Oncology (ASCO) and the Portuguese Society for Medical Oncology; and a role as clinical expert in the area of medical oncology for Infarmed. Ulrike Leiter: relevant financial activities (research support from Merck Sharp and Dohme; speakers and advisory board honoraria from Merck Sharp and Dohme, Novartis and Roche, Sanofi Aventis and travel support from Sun Pharma). All other authors declared to have no conflicts of interest.
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