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000304468 1001_ $$aUgurel, Selma$$b0
000304468 245__ $$aOutcome of systemic therapy in patients with advanced rare skin cancers: A retrospective multicenter DeCOG study of 209 patients.
000304468 260__ $$aAmsterdam [u.a.]$$bElsevier$$c2025
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000304468 520__ $$aFor rare skin cancers, few data exist on the outcome of systemic therapies, particularly immune checkpoint inhibition (ICI). The present study analysed the real-world use of different systemic therapies including ICI, and its outcome in patients with advanced rare skin cancers.This retrospective multicenter study included patients who received systemic therapy for advanced, non-resectable cutaneous angiosarcoma (AS), Kaposi sarcoma (KS), pleomorphic dermal sarcoma (PDS), or cutaneous adnexal carcinoma (CAC). Study endpoints were best overall response (BOR), progression-free survival (PFS) and overall survival (OS).209 patients (77 AS; 81 KS; 14 PDS; and 37 CAC) from 30 centers were included. As first-line treatment AS and KS patients predominantly received chemotherapy (77.9 %; 63.0 %), while PDS and CAC patients mostly received ICI (64.4 %; 43.2 %). BOR in first-line across all therapy types was 65.5 % in KS, 50.0 % in PDS, 41.6 % in AS, and 10.8 % in CAC. BOR for ICI was 66.6 % for PDS, 58.3 % for AS, 33.3 % for KS, and 4.3 % for CAC, irrespective of treatment line. 1-year PFS rate upon any first-line therapy was 70.7 % for PDS, 45.7 % for KS, 25.6 % for AS, and 18.5 % for CAC (p < 0.001). 1-year tumor-specific OS rate was 97.3 % in KS, 84.2 % in AS, 67.7 % in PDS, and 65.4 % in CAC (p < 0.001).Type and outcome of systemic therapy differed between cancer entities. Efficacy of ICI was high in PDS and AS, moderate in KS, and low in CAC. Patients with advanced CAC revealed an extremely poor prognosis regardless of the type of therapy used.
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000304468 650_7 $$2Other$$aAngiosarcoma
000304468 650_7 $$2Other$$aChemotherapy
000304468 650_7 $$2Other$$aCutaneous adnexal carcinoma
000304468 650_7 $$2Other$$aImmune checkpoint inhibition
000304468 650_7 $$2Other$$aKaposi sarcoma
000304468 650_7 $$2Other$$aPleomorphic dermal sarcoma
000304468 650_7 $$2Other$$aTargeted therapy
000304468 7001_ $$aAbu Rached, Nessr$$b1
000304468 7001_ $$aGambichler, Thilo$$b2
000304468 7001_ $$aMengoni, Miriam$$b3
000304468 7001_ $$aTüting, Thomas$$b4
000304468 7001_ $$aHassel, Jessica C$$b5
000304468 7001_ $$aReschke, Robin$$b6
000304468 7001_ $$0P:(DE-HGF)0$$aLodde, Georg C$$b7
000304468 7001_ $$0P:(DE-HGF)0$$aPlacke, Jan-Malte$$b8
000304468 7001_ $$0P:(DE-HGF)0$$aSchadendorf, Dirk$$b9
000304468 7001_ $$aReinhardt, Lydia$$b10
000304468 7001_ $$aKreuter, Alexander$$b11
000304468 7001_ $$aGschnell, Martin$$b12
000304468 7001_ $$0P:(DE-He78)a229f7724466e7efadf4a1ace1ff8af3$$aUtikal, Jochen$$b13$$udkfz
000304468 7001_ $$aErdmann, Michael$$b14
000304468 7001_ $$aDücker, Pia$$b15
000304468 7001_ $$aHelbig, Doris$$b16
000304468 7001_ $$aFranklin, Cindy$$b17
000304468 7001_ $$aGebhardt, Christoffer$$b18
000304468 7001_ $$aPoch, Gabriela$$b19
000304468 7001_ $$aKähler, Katharina C$$b20
000304468 7001_ $$aWeichenthal, Michael$$b21
000304468 7001_ $$aGutzmer, Ralf$$b22
000304468 7001_ $$aHeinzerling, Lucie$$b23
000304468 7001_ $$aWeishaupt, Carsten$$b24
000304468 7001_ $$aMohr, Peter$$b25
000304468 7001_ $$aThoms, Kai-Martin$$b26
000304468 7001_ $$aLang, Berenice$$b27
000304468 7001_ $$aSchilling, Bastian$$b28
000304468 7001_ $$aHaferkamp, Sebastian$$b29
000304468 7001_ $$aSachse, Michael$$b30
000304468 7001_ $$aWelzel, Julia$$b31
000304468 7001_ $$aJochims, Franziska$$b32
000304468 7001_ $$aRaap, Ulrike$$b33
000304468 7001_ $$aSchley, Gaston$$b34
000304468 7001_ $$aTerheyden, Patrick$$b35
000304468 7001_ $$aDippel, Edgar$$b36
000304468 7001_ $$aPöttgen, Christoph$$b37
000304468 7001_ $$0P:(DE-He78)7bf2f090fe39a6cd6f0bccf5ea2d4fb3$$aBecker, Jürgen C$$b38$$udkfz
000304468 7001_ $$0P:(DE-HGF)0$$aTasdogan, Alpaslan$$b39
000304468 7001_ $$aAmaral, Teresa$$b40
000304468 7001_ $$aNanz, Lena$$b41
000304468 7001_ $$aLeiter, Ulrike$$b42
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